We report a 35-year-old man with hereditary cerebroretinal vasculopathy (CR
V) characterized by retinal microvascular changes and a right frontal intra
cerebral mass lesion that suggested a brain tumor. Histopathologic analysis
of the patient's brain lesion as well as reviewed specimens of the patient
's mother, who had reportedly died of a brain tumor, showed no neoplasia bu
t did show cerebral microvasculopathy. CRV should be considered as a differ
ential diagnosis for patients with intracerebral mass lesions, retinal vasc
ular changes, and a positive family history of "brain tumors."