The first case of a child with an arteriovenous malformation (AVM) associated with moyamoya disease is reported. The patient presented ischemic symptoms and underwent indirect bypass surgery on both sides when she was 5 years old. Four years later she suffered from headache, and a small AVM of the left frontal lobe associated with the moyamoya vessels was detected. Single photon emission computed tomography (SPECT) was performed at age 11 and demonstrated low local cerebral blood flow (CBF) in the left frontal lobe and right temporal lobe, although the revascularization after the bypass surgery seemed to be effective, as judged on pancerebral angiography. We feel that brain ischemia due to the moyamoya disease may have played a causative role in the development of the AVM.