Over the past 6 years we at the Neurosurgery Department in Zürich have had the opportunity to manage increasing numbers of patients, especially children, with Moyamoya angiopathy. With increasing awareness of presence of this angiopathy in Europe the number of referrals from all across Europe is constantly on the increase. We have also been able to readdress the presence of the entity of Moyamoya angiopathy i.e. both the Moyamoya disease and the Moyamoya syndrome in the European population. Thorough presurgical workup is mandatory for evaluation of surgical candidates for the type of effective revascularisation procedure and therefore for their successful management. Apart from scrutinizing the routine yet indispensable presenting symptomatology with clinical examination of the patients, our preoperative diagnostic workup mainly consists of a 6 vessel cerebral angiography, cerebral perfusion studies with HMPAO-SPECT and H(2)15O-PET examinations and transcranial Doppler. Longterm follow-up of these patients is indispensable and of great interest to us in terms of etiology and progression of the disease process as well as the choice of effective revascularisation procedure especially in our European population.