Angiomyolipomas are benign mesenchymal tumors, but those that arise from the small intestine are exceedingly rare. We report on a 48-year-old woman who had an ileal angiomyolipoma, who presented clinically with vague abdominal pain and bloody stool. Small-bowel intussusception was shown on an abdominal computed tomography (CT) scan. We discuss the clinical manifestations and clinicopathological and immunohistochemical findings of this benign tumor which appeared in this rare location.