Giant congenital melanocytic naevus associated with Dandy–Walker malformation, lipomatosis and hemihypertrophy of the leg M. Go ¨ nu ¨ l, S. Soylu, U ¨ . Gu ¨ l, E. Aslan, T. U ¨ nal and G. Ergu ¨l Dermatology Clinic, Ankara Numune Education and Research Hospital, Ankara, Turkey doi:10.1111/j.1365-2230.2008.03191.x Summary A 30-year-old woman presented with congenital hyperpigmented plaques and multiple tumoral lesions. Her left leg was hypertrophic, although the bones were normal. Dermatological examination revealed hyperpigmented macules and plaques with hair on the legs, abdominal and lumbar areas (covering > 60% of the total body surface) and multiple naevi on the face, arms, back and chest. Multiple soft masses of various sizes, some of which colocalized with hyperpigmented plaques, were seen on the trunk and legs. Malignant melanoma was excluded by histopathological examinations of multiple biopsy specimens obtained from the pigmented lesions. Histopathological examination of one of the soft masses showed that it was a lipoma. Cranial magnetic resonance imaging and computed tomography scans showed an enlarged fouth ventricule and vermis hypoplasia (Dandy–Walker malformation; DWM). Neurocutaneous melanosis is a rare combined abnormality of the skin and central nervous system. A few case reports relating to the coexistence of giant congenital melanocytic naevus, lipomatosis and hemihypertrophy have been published in the literature. We report this very rare case of giant melanocytic naevus with lipomatosis, hemihypertrophy of the leg, and DWM. Congenital melanocytic naevi (CMN) are defined as benign naevomelanocytic proliferations that are present at birth. 1–3 Giant congenital melanocytic naevi (GCMN) are seen rarely, and are important because of the possibility of malignant transformation, aesthetic con- cerns, and association with other abnormalities, most commonly in the central nervous system (CNS). Neuro- cutaneous melanosis (NCM), is a rare combined abnormality of the skin and CNS. 4 We present an uncommon case with the association of GCMN and NCM, lipomatosis, hemihypertrophy of the left leg, and Dandy–Walker malformation (DWM). Report A 30-year-old woman presented with a 1-year history of headache, nausea and vomiting. She had hyperpig- mented naevi and soft masses on her body that had been present since birth. There was no consanguinity in her family and no members of the family had similar lesions. Physical examination revealed hemihypertrophy of the left leg; the circumference of the right cruris was 320 mm, left cruris 390 mm, right thigh 307 mm and left thigh 480 mm. The lengths of the right and left legs (between the medial malleolus and anterior superior iliac spine) were 800 mm. Hyperpigmented hairy plaques were seen on the patient’s legs, buttocks, abdomen and lumbar area, covering 60% of the total body surface. There were many melanocytic naevi of various sizes on the scalp, face, back, chest and arms. Multiple soft masses, also of various sizes, some of which Correspondence: Dr Mu ¨ zeyyen Go ¨ nu ¨ l, Barıs ¸ Mah, Gu ¨ zelyaka Cad, Demet- kent sit, B-2 Blok No. 44 Yenimahalle, Ankara, Turkey. E-mail: muzeyyengonul@yahoo.com Conflict of interest: none declared. Accepted for publication 5 September 2008 Clinical dermatology • Concise report Clinical and Experimental Dermatology Ó 2009 The Author(s) e106 Journal compilation Ó 2009 British Association of Dermatologists • Clinical and Experimental Dermatology, 34, e106–e109