PURPOSE: To demonstrate the MRI findings of olivopontocerebellar atrophy. MATERIALS AND METHODS: We retrospectively reviewed the MRI findings of eight patients who had been diagnosed by clinical manifestation and the peculiar pattern of atrophy and signal change on MRI. RESULTS: Seven patients had an atrophy of the olive, pons and cerebellum and increased signal change of the transverse pontine fiber, median raphe and middle cerebellar peduncle on T2WI. Of these, six patients had severe atrophy of the olive, pons and cerebellum and decreased signal change of the basal ganglia, red nucleus, substantia nigra or dentate nucleus on T2WI. Additionally, four of six patients had a cerebral atrophy. Except one patient who had an urinary incontinence, these 5 patients had not been associated with extrapyramidal or autonomic symptom. The other patient with relatively short duration of the disease had only cerebellar atrophy without signal change on T2WI . CONCLUSION: With progressing of the olivopontocerebellar atrophy, cerebral atrophy and decreased signal change of the basal ganglia, red nucleus, substantia nigra or dentate nucleus on T2WI is combined. Thus, MRI is essential in establishing the diagnosis and evaluating the severity of olivopontocerebellar atrophy.