We report a case of large choroid plexus papilloma of the fourth ventricle in a 23-year-old woman. She presented with severe headache, dysphagia, and gait disturbances. Horizontal nystagmus, ataxic gait and quadriparesis were detected on initial examination. Imaging studies showed a large mass in the left side of brain stem and a marked hydrocephalus. The tumour was removed by microsurgical dissection of the cerebellomedullary fissure. We have discussed the effectiveness of this approach for removal of bulky tumors of the fourth ventricle and reviewed the literature about its benefits and potential hazards.