A novel presentation of Hecht-Beals trismus pseudocamptodactyly syndrome: Review of literature and holistic management Partha Chattopadhyay, Cyriac Abby Philips, Debargha Dhua, Prabir Kumar Josh, Prasanta Mukhopadhyay ABSTRACT Introduction: Hecht-Beals Trismus Psuedocamptodactyly syndrome, a rare autosomal dominant inherited disease encompass a variety of genotypic and phenotypic verges that affects members of a family. Case Report: Here we report a patient, who presented with congenital coal black hyperpigmentation of the skin with progressive trismus. Conclusion: With emergence of new reports, the mutational and expressive variations of this disease is becoming more enigmatic. Our tidings shed light on a novel clinical association with coal black skin pigmentation in whom trismus was dealt successfully through operative methods. Further mutational analysis and clinical probing would help in concreting a definite categorization of this diseases that lie between these gamuts. Keywords: Trismus, Masseter Spasm, Hypermelanosis ***** Chattopadhyay P, Philips CA, Dhua D, Josh PK, Mukhopadhyay P. A novel presentation of Hecht-Beals trismus pseudocamptodactyly syndrome: Review of literature and holistic management. International Journal of Case Reports and Images 2012;3(3):19-23. ********* doi:10.5348/ijcri-2012-03-101-CR-6 INTRODUCTION Trismus is a condition in which there is inability to normally open the mouth due to varied reasons, commonly tetanus, sub mucosal fibrosis, condylar fracture, temporo-mandibular joint dysfunction, neuroleptic syndromes, peritonsillar abscess and ankylosis of temporomandibular joint. Trismus can also be part of a rare syndrome complex known as the Trismus-Psuedo-Camptodactyly Syndrome [1]. Here we present a patient who presented to us with Trismus- Psuedo-Camptodactyly Syndrome, in whom congenital progressive diffuse coal black hyperpigmentation was a striking feature, with mild affection of his elder brother (coal black pigmentation to a lesser degree with mild trismus). Corrective surgical procedure of the trismus and microstomia resulted in betterment and quality of life of our patient without any recurrence even after nine months. CASE REPORT A 17-year-old male, presented to us with progressive difficulty in opening the mouth for two years, associated with bony deformities of skull and extremities and www.ijcasereportsandimages.com