Angiomyolipomas(AMLs),a form of benign mesenchymal hamartoma,arise primarily in the kidneys of patients with or without tuberous sclerosis.Extra-renal AMLs are very rare and are most commonly found in the liver. AMLs of the small intestine are exceedingly rare.Here, a case of a 28-year-old man,who presented with ileal intussusception caused by ileal AML is reported.The clinicopathological and immunohistochemical findings of ileal AMLs are discussed and the literature on small intestinal AMLs is reviewed. Angiomyolipomas (AMLs), a form of benign mesenchymal hamartoma, Anticokes into the kidneys of patients with or without tuberous sclerosis. Extr-renal AMLs are very rare and are those most commonly found in the liver. AMLs of the small intestine are exceedingly rare. Here, a case of a 28-year-old man, who presented with ileal intussusception caused by ileal AML is reported. Clinicopathological and immunohistochemical findings of ileal AMLs are discussed and the literature on small intestinal AMLs is reviewed.