A 36-year-old female had a giant congenital pigmented nevus on her anterior trunk. She complained of muscle weakness of the right arm and leg in August 1988, and she was admitted to our hospital to take medical examinations in September 1988. Neurological examination revealed right hemiparesis and exaggerated deep tendon reflexes. Three small falx meningiomas were shown on CT scans and MR images, but they did not appear to be causes of her symptoms. CT-myelography and MR images revealed an intradural extramedullary tumor spreading from the foramen magnum to C2. Oncotomy and C1 laminectomy were performed. Histological finding was compatible with transitional meningioma. Neurological symptoms markedly improved after operation. This patient is considered as a case of neurocutaneous syndrome with central nervous system tumors. Considering that both giant pigmented nevus and meningioma originate from neural crest, they appear to be closely related each other. Such a combination of giant pigmented nevus and meningioma has not yet been reported.