Hereditary nevoid basal cell carcinoma (BCC) syndrome, known as Gorlin syndrome is a rare autosomal dominant familial tumor syndrome characterized by more than 30 congenital malformations.1 Affected individuals often present with recurrent BCCs that slowly progress and cause severe disfigurement despite treatment with surgery, curettage and electrodesiccation, cryosurgery, dermabrasion, laser vaporization, intralesional interferon, and topical fluorouracil. We describe a patient with Gorlin syndrome for whom systemic therapy was needed to arrest the progression of aggressive BCCs.