Moyamoya disease is a cerebral vasculopathy of unknown etiology frequently seen in the Asian population. We report a case of moyamoya vasculopathy in an African-American child who had renal failure followed by cerebral ischemia. Our patient presented with hemolytic uremic syndrome (HUS) and renal failure, and later developed seizures. We believe that in this patient HUS led to the pathogenesis of moyamoya disease. We suggest that patients with HUS who develop any neurological symptoms should be investigated for moyamoya vasculopathy for early diagnosis and treatment.