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BACKGROUND The de novo occurrence of renal artery stenosis in renal arteries that were angiographically confirmed to be normal in the past has never been reported before in patients with moyamoya disease. CASE DESCRIPTION During the long-term follow-up of pediatric patients with moyamoya disease, we observed 3 patients who developed de novo renal artery stenosis in arteries that had been angiographically confirmed to be normal 1 year after the surgery (7 years on average, ranging from 4 to 11 years). All of these patients were neurologic