A case of acoustic neurinoma with huge cysts was reported clinico-pathologically. A male developed at the age of 30, the left-sided hearing difficulty. At the age of 48, he noted the staggered gait which after a while almost entirely cleared up. At the age of 54, he was again unable to walk because of ataxia. Neurological symptoms at the admission to hospital were almost agreed with those in acoustic neurinoma. The symptoms showed complete remission partly because of medication, but they became worse rapidly again, The patient died from bronchial pneumonia at the age of 60. Autopsy disclosed a tumor with huge cysts at the left cerebello-pontine angle which was diagnosed neurinoma microscopically. The most unusual clinical point lies on the course. It lasted thirty long years and moreover displayed an intermittent course showing remission and exacerbation. Another point of interest is the huge cysts found in the tumor at autopsy. The authors discussed the cases manifestating atypical clinical course and tumors with huge cysts in the literature.