A 23-year-old woman with basal cell nevus syndrome (BCNS), or Gorlin's syndrome, was given the diagnosis at age 2 years of a medulloblastoma that was resected and treated postoperatively with craniospinal irradiation. Multiple basal cell carcinomas developed at the craniospinal irradiation port site 8 years later. She subsequently developed multiple meningiomas within the area of irradiation at age 20 years. This case reviews early presentation of BCNS, newly described differences between medulloblastomas in patients with BCNS and nonsyndromic medulloblastomas, and global assessment of patients by the treating dermatologist of this patient population. This is the first report in the dermatologic literature, to our knowledge, of radiation-induced meningiomas in a patient with BCNS.