目的　报告一橄榄 -桥脑 -小脑萎缩 (OPCA)伴视网膜变性家系的临床特征。方法　对先证者进行临床、电生理和头颅 MRI检查 ,并对家系遗传规律进行分析研究。结果　此家系 4代中 8例罹患 ,呈常染色体显性遗传 ,存在早现现象。临床表现为进行性视力下降、视网膜变性、眼球运动障碍和小脑性共济失调。先证者视网膜电图示潜伏期延长 ,视觉诱发电位 (VEP)、体感诱发电位 (SEP)和脑干听觉诱发电位 (BAEP)异常 ,事件相关电位 P3 0 0 和 N40 0 正常 ;MRI示桥脑、橄榄体和小脑萎缩。结论　 OPCA伴视网膜变性呈常染色体显性遗传 ,呈早现现象。临床主要表现为小脑性共济失调、进行性视力下降、眼肌麻痹和视网膜变性。 Objective To report the clinical features of an olivopontocerebellar atrophy (OPCA) pedigree with retinal degeneration. Methods The probands were examined by clinical, electrophysiological and cranial MRI, and the genetic laws of the pedigrees were analyzed. Results The family of 4 generations in 8 patients suffering from, was autosomal dominant inheritance, there is an early phenomenon. Clinical manifestations of progressive vision loss, retinal degeneration, eye movement disorders and cerebellar ataxia. The probands showed prolonged latency, visual evoked potential (VEP), somatosensory evoked potential (SEP) and brainstem auditory evoked potential (BAEP) abnormalities, with event-related potentials P3 0 0 and N40 0 normal. MRI showed pons, Olive and cerebellum atrophy. Conclusion OPCA with retinal degeneration was autosomal dominant, premature appearance. Clinical manifestations of cerebellar ataxia, progressive visual loss, ophthalmoplegia and retinal degeneration.