In 1972, Neault and his co-workers reported seven cases of uveitis associated with intracranial reticulum cell sarcoma. Recently we have experienced a similar case for the first time in Japan. A 32-year-old woman registered on March 10, 1971, complaining of blurred vision in the left eye for about two weeks. By ophthalmic examination, left posterior uveitis was diagnosed but the etiology was unknown. Treatment with corticosteroids was begun, but her left eye continued to fail in spite of the treatment. In August, 1971, she complained of weakness of left arm and leg, and in October, she suffered from severe headache and vomiting. At that time, uveitis appeared in the right eye too. Neurological findings and carotid angiogram indicated a right cerebral lesion. On November 5, 1971, a right frontoparietal craniotomy was performed but no tumor was found. Since then her neurological and eye symptoms had been progressively worse. The patient died on July 12, 1972. Postmortem examination revealed the tumor infiltrating in the bilateral diencephalon, left internal capsule, left lenticular nucleus, left temporal lobe, midbrain, pons, left dentate nucleus, optic chiasma and intracranial portion of the optic nerves. But no tumor was found at any other parts of the body. Histologically the tumor was a reticulum cell sarcoma. The eyeballs were not examined histologically, but the uveitis in this case was thought to be closely related to the intracranial reticulum cell sarcoma. If the uveitis is resistant to the treatment, we must consider a possibility of reticulum cell sarcoma of the brain.