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Nevoid basal cell carcinoma or Gorlin–Goltz syndrome is an autosomal dominant inherited disorder that results in numerous basal cell carcinomas (BCC) as well as a number of other facial and skeletal findings. The estimated prevalence varies from 1/57000 to 1/256000 with a male-to-female ratio of 1:1. Main clinical manifestations include multiple BCCs, odontogenic keratocysts of jaws, intracranial ectopic calcifications, and skeletal abnormalities affecting the ribs, vertebra, and skull. Management requires a multidisciplinary approach, with surgical removal of odontogenic keratocysts; surgery for BCCs should be indicated when the number of such lesions is limited. We report a case that was managed surgically, with a follow-up of more than 2 years having no signs of recurrence and relevant literature review.