ISSN 2320-5407 International Journal of Advanced Research (2016), Volume 4, Issue 5, 750-753 750 Journal homepage:http://www.journalijar.com INTERNATIONAL JOURNAL Journal DOI:10.21474/IJAR01 OF ADVANCED RESEARCH RESEARCH ARTICLE Title: A Dental Perspective on the Rare Disorder of Acromesomelic Dysplasia - A Case Report. Dr. Fehan Ahmed Khan,Dr.Gaurav Kumar Mittal,Dr.Adnan Ahmed,Dr.Deepanshu Kumar Nigam, Dr.SoorajSomen, Dr. HansikaPahuja and Dr.Sorav Gupta. Manuscript Info Abstract Manuscript History: Received: 16 March 2016 Final Accepted: 19 April 2016 Published Online: May 2016 Key words: Acromesomelic dysplasia, rare disease, dental management, AMD *Corresponding Author Dr. Fehan Ahmed Khan. Introduction: Acromesomelic dysplasia, the Maroteaux type is listed as a “rare disease” by the office of rare diseases (ORD) of the National Institute of Health (NIH). This means that acromesomelic dysplasia, Maroteaux type (AMDM), or a subtype of acromesomelic dysplasia affects less than 200,000 people. Acromesomelicdysplasiasare skeletal disorders that disproportionately affect the middle and distal segments of the appendicular skeleton. As with the appendicular features, the vertebral features become more pronounced throughout childhood. The pelvis has a typical configuration, and the cranium may demonstrate frontal bossing, with a normal head circumference, hypoplastic maxilla and malocclusion. Case report:The following case report discusses the medical and dental findings of acromesomelic dysplasia and their subsequent management. Conclusion: Disorders like acromesomelic dysplasia have rarely been documented for any oral findings. Further documentation of such cases would be essential in establishing the full scope of the oral features. The findings enlisted here suggest that early preventive measures and intercepting any developing malocclusions would suffice the promotion of good oral health during infancy and childhood. Copy Right, IJAR, 2016,. All rights reserved. Introduction:- Acromesomelic dysplasia Maroteaux-type (AMDM), first described by Maroteaux in 1971, is a rare autosomal recessive osteochondrodysplasia. 1 This disorder is characterized by acromelia and mesomelia. Mesomelia describes the shortening of the bones of the forearms and lower legs relative to the upper parts of those limbs. Acromelia is the shortening of the bones of the hands and feet. Thus, the short stature of affected individuals is the result of unusually short forearms and abnormal shortening of bones of the lower leg. 2 About 50 cases of AMDM have been reported till date, most of them being the classical variety. 3 This is an autosomal recessive skeletal dysplasia with a prevalence of ~1/1,000,000. 4 A diagnosis of AMDM type is usually made on the basis of combined clinical, genetic and radiographic findings. Although this condition is usually diagnosed at birth and becomes more obvious in the first two years of life. Recently, Kant et al reported the mapping of a gene for AMDM to human chromosome 9p13-q12 by homozygosity mapping in four inbred families. 5 Birth lengths and weights are normal, although mild shortening of long bones may be detected in some affected infants. 4 In patients with AMDM, all skeletal elements are present, but they have abnormal rates of linear growth (Langer and Garrett 1980). 6 By two years of age, radiographic skeletal changes are diagnostic for AMDM and include abnormal growth plates and short, misshapen bones in the extremities. 4 Here in, we are reporting a case of a child affected with AMDM, along with dental findings and management. Case report:- A seven year old female child reported to our department, with the chief complaint of dental caries in relation to upper anterior and lower posterior teeth. Child showed stunted growth with normal intelligence. Previous medical records showed that she was diagnosed with AMDM at Indira Gandhi Institute of Child Health, Karnataka. Family