Loss of the tumor suppressor von Hippel-Lindau (VHL) gene is an established genetic risk factor for renal cell carcinoma (RCC) and the affected individuals are at higher risk to develop tumors including pheochromocytomas, retinal angioma, pancreatic cysts and central nervous system hemangioblastomas [1], [2]. Here, VHL is linked to hemangioma of retina.