Objective: to present a clinical case of spontaneous obliteration of congenital arteriovenous malformation of the brain in a 4-year-old child. Neurosonography revealed arteriovenous malformation in a 1-month-old girl; the diagnosis was confirmed by magnetic resonance imaging and multispiral computed tomography of the brain. The child did not receive surgical and drug treatment. Dynamic observation showed a decrease of malformation, and a control examination (age: 4 years) revealed a focus of gliosis, which indicated a complete spontaneous regression of arteriovenous malformation. The authors discuss diagnostic features, tactics of conservative and surgical treatment of children with arteriovenous malformation.