The association of moyamoya syndrome and congenital heart disease is described in 5 patients at Children's Hospital, Boston, MA. Stroke was the presenting sign in 3 and seizures in 2. Coarctation of the aorta with septal or valvular defects was diagnosed in 3, and tetralogy of Fallot in 2. Moyamoya, diagnosed after surgery for congenital heart disease, was treated by cerebral revascularization. 
COMMENT. Moyamoya syndrome should be considered in the differential diagnosis of stroke or seizures associated with congenital heart disease, both before or after surgery.