A case of pituitary adenoma associated with multiple neurinomas in the central nervous system was presented. A 52-year-old man was referred to us for surgical treatment of an intradural extramedullary cervical cord tumor. He had been operated on for a cauda equina tumor when he was 43 years old, and again for a glossopharyngeal neurinoma at 49 years of age. His brother expired in childhood, and had multiple subcutaneous nodules. The patient had been complaining of left leg pain, left shoulder pain, and hypesthesia of the right leg and foot. Examination showed 6th cervical nerve root sign. MRI revealed a well-circumscribed extramedullary tumor at the C5 level, and, in addition, incidentally showed an intra- and supra-sellar tumor which was isointensity on T1 weighted and high intensity on T2 weighted images. Myelography showed multiple extramedullary tumors in the lumbar region. Endocrinological study revealed an increased serum prolactin level (818.0ng/ml). The patient had neither café au lait spots nor subcutaneous nodules. A neurinoma of C6 root was totally removed and chromophobe pituitary adenoma was partially removed through a transsphenoidal approach. Neurofibromatosis is known to be associated with many kinds of tumors in the central nervous system. They are usually neurinomas, meningiomas or gliomas, and association of pituitary adenomas has been reported in only three cases, one of which being a prolactin secreting adenoma. Coexistence of multiple primary brain tumors has been also reported apart from phakomatosis. The most common combination is association of glioma and meningioma, and it is probably incidental coexistence due to their high frequency.(ABSTRACT TRUNCATED AT 250 WORDS)