Moyamoya disease is an extremely rare cerebrovascular condition that predisposes affected patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches. To our knowledge, this is the first report of a lethal complication due to moyamoya disease after septorhinoplasty. A 25-year-old female Caucasian patient presented to our outpatient clinic with impaired nasal breathing for septorhinoplasty. Regrettably the patient died 6 days postoperatively due to progressive infarct series affecting all major cerebral vessels. Despite a thorough knowledge of possible local complications after septorhinoplasty, it is of utmost importance to consider rare general complications like moyamoya disease. Although cerebral infarctions are very rare in young people, it is crucial to identify and correctly interpret underlying typical symptoms.