Angiomyolipoma (AML) is a rare, benign tumor composed of thick-wall blood vessels, smooth muscle component and mature adispose tissue. Differentiation from other benign and malignant mesenchymal lesions of oral cavity depends on recognition of these three histologic components, and immunohistochemical (IHC) techniques are also helpful. This tumor arise from perivascular epithelioid cells (PEComas) and kidneys followed by liver are the main locations of this soft tissue tumor. AMLs are rarely found in oral cavity and few case reports of oral AML have been reported in the literature. We report the first case of concurrent occurrence of AML of the tongue and peripheral giant cell granuloma of the gingiva in a 59-year-old patient. Clinically it was presented as a painless nodular mass with a smooth surface on the dorsal of the tongue. Based on histopathologic features and IHC staining the diagnosis of oral AML was done. The other lesion was a small sessile mass in interdental papillae of the lower incisors and microscopic examination showed the histologic features of a peripheral giant cell granuloma. Concurrent occurrence of PGCG which is a reactive mucosal hyperplasia and AML in our patient, could show the probable role of local trauma in the pathogenesis of these lesions.