Objective: To evaluate the clinical features and MRI value in the diagnosis of olivopontocerebellar atrophy (OPCA). Methods: 8 cases OPCA confirmed by clinic and MRI were reviewed. Results: All patients were sporadic cases. Images of all cases showed atrophy of the brainstem and cerebellar hemisphere. The basis pontine "cross sign" were found in 6 Cases on T2 weighted images. Conclusion: The possibility of OPCA should be exclude by early MRI in patients presenting with cerebellar ataxia or extrapyramidal symptoms.