✓ The authors describe a rare case of idiopathic distal lenticulostriate artery (LSA) aneurysm in a 5-year-old boy who presented in the emergency department with a sudden onset of headache. Admission computed tomography scans revealed an intracerebral hemorrhage in the left caudate nucleus with intraventricular extension. Angiographic studies demonstrated a left medial LSA aneurysm. The patient underwent a left parasagittal frontal craniotomy, the lateral ventricle was accessed via the anterior transcallosal approach, and the aneurysm was removed after sectioning of the parent vessel. The child left the hospital after 5 days; at that time he was asymptomatic and without motor impairment. The optimum treatment of aneurysms involving small perforating arteries is controversial and depends mainly on the causative factors. The pathogenesis and treatment of these unusual aneurysms are discussed.