Between January 1941 and June 1989, 46 children below the age of 18 with an arteriovenous malformation (AVM) were managed. There were 7 patients with AVM diagnosed before the age of 2; 10 patients were diagnosed between the ages of 3 and 10; and 29 patients were diagnosed between 11 and 18. There were equal numbers of male and female patients. Twenty-five of the AVMs were large (greater than 5 cm longest diameter). All 7 AVMs diagnosed before the age of 2 were large. The usual clinical presentation was congestive heart failure, bruit and an enlarging head. Three patients underwent excision with 2 deaths and 1 excellent result. In 11 patients (aged 3-18) with AVM without history of hemorrhage, 3 had excision with 2 excellent and 1 fair result. Four remained stable. Four developed progressive deficits or hemorrhage. In 10 patients (aged 3-18) with AVM and hemorrhage who were treated medically, 7 (70%) had an episode of re-hemorrhage. Three patients had excision of AVM after re-hemorrhage, but before the age of 18 with an excellent result. Eighteen patients (aged 3-18) with AVM and a single episode of hemorrhage underwent excision with 17 excellent or good results and 1 fair result. The overall mortality was 7%. Eighty-five percent of the children with excision of AVM had an excellent or good result. The best treatment for AVM in children is surgical excision.