Abstract: Objectives: Von Hippel Lindau disease is a rare, autosomal dominant neoplastic syndrome characterized by multiple benign and malignant tumors that affect multiple organs, including the central nervous system (CNS) hemangioblastomas, visceral tumor, and cysts. The average age of onset ofVon Hippel Lindau disease is between 26.3 years old and 30.9 years old, with an incidence rate of 1 : 36 000. This disease has a poor prognosis, and the average life expectancy is below 49 years old. The main causes of death is usually rupture and bleeding of CNS hemangioblastomas, renal cell carcinoma, and malignant hypertension caused by pheochromocytoma. We present a case ofVon Hippel Lindau disease with CNS hemangioblastoma, complicated by bilateral renal cancer, and multiple cysts in the epididymis, liver, and pancreas in the First Affiliated Hospital of Huzhou Teachers College. The patient underwent nephron sparing surgery of the left kidney and he was given sunitinib malate as molecule targeted therapy. Patient followed-up consistently for 30 months, and the patient survived and is living well.