Abstract Gorlin-Goltz syndrome is characterized by nevoid basal cell carcinomas, odontogenic keratocysts of the jaws, palmar and plantar pits, falx calcifications, and various cancer susceptibilities attributed to a mutation in a tumor suppressor gene. We present an 11-year-old female with Gorlin-Goltz syndrome. A medulloblastoma was diagnosed when she was 4 years old. She received total excision of the tumor and adjuvant chemotherapy and radiotherapy. One year later, the tumor relapsed with cerebral spinal fluid seeding. She recovered spontaneously with no treatment, and she has not developed basal cell carcinoma within 6 years. This patient with Gorlin-Goltz syndrome had a good prognosis with regard to the medulloblastoma. Diagnosis of the syndrome should be made as early as possible to avoid aggressive radiotherapy that may induce basal cell carcinomas.