A 15-year-old girl was diagnosed with a spinal anaplastic pilocytic astrocytoma. The histologic features were similar to pilocytic astrocytoma WHO grade I, but with an increased mitotic rate, high nuclear pleomorphism, microvascular proliferation, and necrosis. The tumor was subtotally resected and treated with chemotherapy and irradiation. Four years after diagnosis no local progression was seen. At the age of 17, the patient developed a supratentorial primitive neuroectodermal tumor. We report the first case of a primary anaplastic pilocytic astrocytoma and primary primitive neuroectodermal tumor occurring in the same patient.