Angiomyolipoma (AML) is a benign tumor that usually affects the kidney. Extrarenal AML is
uncommon. Intraoral AML is extremely rare and only 13 cases have been reported in the
English-language literature. Although oral AML shares the terminology with renal AML, it presents as
solitary mass not associated with tuberous sclerosis complex and is negative for human melanoma
marker HMB-45. This article reports a rare case of intraoral AML presenting on the hard palate of
a 52-year-old male patient. The surgical excision of this lesion was considered curative and
no recurrence has been documented. The literature concerning intraoral AML is also reviewed.
(CU Dent J. 2017;40:63-70)