von Hippel-Lindau disease with bilateral multiple renal cell carcinoma managed by right radical nephrectomy and left repeat partial nephrectomy.

von Hippel-Lindau (VHL) disease is an autosomal dominant disorder characterized by cysts and cystadenoma in the kidney, pancreas and epididymis and angiomas of the central nervous system and retina as well as renal cell carcinoma (RCC), phaeochromocytoma, islet tumors of the pancreas, and endolympatic sac tumors. VHL for its multicentric-characteristic and bilateralism often puts the surgeon in challenging situation. We present a case of VHL with bilateral RCC and retinal angiomas managed with right radical nephrectomy and left repeat partial nephrectomy.