Intrasellar arachnoid cyst is very rare. We report a case of intrasellar arachnoid cyst. A 44-year-old male was admitted for evaluation of his headache and visual disturbance on August 6, 1993. Neurological examination revealed bilateral decreased visual acuity and visual field defect. Endocrinological examination showed panhypopituitarism. Other neurological findings were normal. X-ray film of the skull showed a ballooning dilation of the sella turcica with thinning of the sellar floor. CT scan showed a cystic lesion with CSF-density occupied the sella. After intravenous administration of contrast medium, the cyst showed no enhancement. MRI showed the intrasellar mass had the same characteristics as the surrounding subarachnoid space. Bilateral carotid angiographies demonstrated that the carotid siphons were stretched and displaced laterally, and the A1 portions of the anterior cerebral arteries were raised. We made a diagnosis of intrasellar cystic lesion. On August 18, the sella turcica was opened via the transsphenoidal rhinoseptal approach. The cyst contained CSF-like fluid, and a part of the cyst wall was resected. The cavity was filled with Gelfoam and the sellar floor was repaired with bone flap. Postoperatively, the patient's visual disturbance improved, but diabetis insipidus appeared and required hormonal replacement. The patient was discharged on September 27 with improvement of visual acuity and visual field. Histological examination demonstrated that the cyst wall consisted of thick arachnoidal cells with fibrous connective tissue. The arachnoidal cells with oval nuclei was stained with epithelial membrane antigen. Symptoms, signs and radiological findings of intrasellar arachnoid cyst are similar to those of various sellar lesions including pituitary adenoma, craniopharyngioma, empty sella, Rathke's cleft cyst, epidermoid et al.(ABSTRACT TRUNCATED AT 250 WORDS)