Gadolinium-enhanced MRI was used to determine the frequency and distribution of CNS lesions in 50 patients with Von Hippel-Lindau Disease (VHL) at the National Institutes of Health, Bethesda, MD; University of Louisville, KY; and the Massachusetts General Hospital, Boston, MA. CNS hemangioblastomas were found in 36 (72%). The cerebellum was affected in 52%, spinal cord in 44%, and the brainstem in 18%. One-half of these tumors were asymptomatic and without clinical signs. The youngest patient was 11 years old and asymptomatic. The youngest symptomatic patient with VHL was 2 years of age with symptoms of ocular lesions. The mean age of all VHL patients was 34 years with a range of 11-62 years. 
COMMENT. Hemangioblastomas and VHL may be detected by 11 years of age and the screening of patients at-risk for VHL should begin at 11 years using gadolinium-enhanced MRI; ophthalmic examination should be initiated within the first two years of life. Diagnosis of VHL is important for genetic counseling as well as early detection of unsuspected ocular and visceral tumors which may lead to blindness or early death unless treated.