The disease moyamoya is rare in Europe, but it is seen more frequently in Japan and other Asian countries. Moyamoya is characterized by progressing occlusion of the cerebral arteries and secondary development of pathological collateral vessels induced by ischaemia. This case report describes a newly diagnosed patient with moyamoya, who was referred to a paediatric clinic because of headache and involuntary movements and sounds. Due to the rare occurrence of moyamoya, the purpose of this case report is to draw attention to its diagnosis with a view to achieving an early diagnosis of the disease.