report a case of a 30-year-old Japanese female patient presenting a painless mass of left lower lip, pathologically diagnosed as angioleiomyoma. The surgically removed mass was a well-encapsulatedtumor, and measuring approximately 5 mm x 10 mm. Histologically, the tumor was composed of vascularspace surrounded by smooth muscle like cells. Immunohistochemically, the tumor cells were positive for alpha-smooth muscle actin (SMA), desmin and vimentin and not positive for S-100 protein. The endotheliumof the vessels were positive for Factor VIII related antigen, and not positive for S-100 protein. Postoperativeobservation has revealed no recurrence as of 5 years 6 months. A review of the English literature revealedthat 158 cases which documents angioleiomyoma of the oral cavity, not including the present case. Themean age in 136 of 158 was 47.9 years, and a range from 3.5 to 85 years, and yielding a male to femaleratio was 1.66: 1. The most frequency reported site was the lip, in 47.1%. c 2012 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rightsreserved.