In recent years advances have been made in the clinical and genetic aspects of von Hippel-Lindau disease (VHL). Retinal capillary hemangioma is the most common manifestation of VHL disease and, therefore, ophthalmologists are frequently involved in the care of patients with this disease. The incidence of VHL disease is approximately 1 in 40,000 live births. It is estimated that there are approximately 7000 patients with VHL disease in the USA. The inheritance of VHL disease is autosomal dominant with high penetrance. Depending on the clinical circumstances, retinal capillary hemangioma may be managed by observation, laser photocoagulation, cryotherapy, and plaque radiotherapy. Typical extraocular lesions associated with VHL disease are central nervous system hemangioma, renal cyst, renal carcinoma, pancreatic cysts and adenoma, pancreatic islet cell tumors, pheochromocytoma, endolymphatic sac tumor of the inner ear, and cystadenoma of the epididymis and the broad ligament. The life expectancy of affected individuals may be improved by early detection and treatment of varied manifestations with the use of surveillance protocols. Identification of the VHL gene on chromosome 3p25-26 has now made it possible for suspected individuals to undergo genetic testing with a high degree of accuracy. We review herein the ophthalmic manifestations and treatment of retinal capillary hemangioma and systemic findings of the VHL disease.