Nevoid basal cell carcinoma – A case report with familial manifestation

Nevoid basal cell carcinoma syndrome is a rare genetic disorder characterised by multi-organ abnormities such as odontogenic keratocysts in the jaw, skeletal abnormities and multiple basal cell carcinoma etc. Herein, we report a case of nevoid basal cell carcinoma in a 10 year old boy with multiple odontogenic keratocyts of jaw, syndactyly on the left hand, palmar and plantar pits, frontal bossing and whose mother and younger brother also have clinical manifestations of the syndrome. No evidence of basal cell carcinoma seen.

Keywords: Nevoid basal cell carcinoma, Gorlin-Goltz syndrome, Odontogenic keratocyst, KCOT, Multiple OKC.