A questionnaire was distributed in early 1996 to 160 leading European neurological, neuro-pediatric and neurosurgical centers to assess the present status of Moyamoya disease in Europe. The response rate was 43%. Information was obtained on a total of 168 patients, of whom 110 had presented before 1992, and 58 from 1993 onward. 82% of the patients were Caucasian. In all other respects, the clinical findings were similar to those observed in Japan. The present study yields an incidence of 0.3 patients per center per year, which is approximately one-tenth of the incidence in Japan. Alongside these results, the history of the recognition and treatment of this disease in Europe is briefly discussed.