A previously healthy 61-year-old man presented with basal ganglia hemorrhage caused by rupture of a small aneurysm arising from the distal lenticulostriate artery associated with moyamoya-like disease and manifesting as left hemiparesis and dysarthria. The patient underwent frontotemporal craniotomy. Neck clipping of the aneurysm was performed through the hematoma cavity. He was transferred for rehabilitation with left hemiparesis. Three-dimensional computed tomography angiography was very useful for preoperative planning and evaluation of the anatomical correlation between the aneurysm and the hemorrhage.