A female infant manifested a rare case of spontaneous regression of a vascular malformation in the occipital lobe after removal of another arteriovenous malformation in the frontal lobe. She was born with multiple nevi on the face, body, and upper and lower extremities. She demonstrated developmental retardation at 8 months of age. Computed tomography at 11 months of age demonstrated ventricular enlargement and a mass in the subdural portion of the left anterior fossa. Magnetic resonance images demonstrated signal void signs in the left frontal lobe, which suggested vascular malformation. Cerebral angiograms disclosed two vascular malformations. The malformation in the frontal lobe was totally removed. Cerebral angiograms 25 days after the operation failed to demonstrate either vascular malformation previously observed. Hemodynamic change following the removal of the arteriovenous malformation may have contributed to the occlusion of the remaining malformation.