Basal cell nevus syndrome and medulloblastoma: a case report.

The basal cell nevus syndrome (BCNS) is a rare autosomal dominant disorder characterized by multiple nevoid basal-cell carcinomas of the skin, recurrent odontogenic keratocysts, skeletal anomalies, developmental malformations, and the predisposition to other neoplastic processes. Among the well known relationships of the syndrome with cancer is that of medulloblastoma. The first known case of BCNS with medulloblastoma in Puerto Rico is presented.