Angiomyxolipoma is a rare tumor characterized by a proliferation of adipose tissue associated with a myxoid stroma and multiple vascular channels. To date, ten cases of angiomyxolipoma located in subcutaneous tissue, spermatic cord and subungual area have been reported in the literature. We describe the clinical and histopathologic findings of the first case of intraoral angiomyxolipoma in a 12 year-old male, as well as the utility of immunohistochemistry as a useful tool in differential diagnosis of this particular lesion with respect to other benign and malignant lesions derived from adipose tissue.