Von Hippel-Lindau disease, one of the phakomatoses, is believed to be a disorder of mesodermal differentiation. Renal lesions, usually cysts or adenocarcinomas with an occasional hemangioblastoma, occur in approximately two thirds of all patients. The renal neoplasms previously reported have been multiple, bilateral, and usually beyond resection. A thirty-eight-year-old white male with a cerebellar hemangioblastoma and bilateral renal adenocarcinoma underwent suboccipital craniotomy, right heminephrectomy, and left radical nephrectomy. No evidence of recurrent disease can be identified ten months postoperatively. An aggressive approach in this systemic disease appears to be warranted.