Angiomyolipoma (AML) in the oral cavity is extremely rare. Their histological features are characterized by admixtures of blood vessels, smooth muscles and mature adipose tissues. We report a case of sporadic AML in the lower lip of a 56-year-old man. Clinically, it presented as a mucocele-like lesion. An excised specimen showed a well-circumscribed, 1.3 cm × 1.0 cm × 0.8 cm-sized, yellowish mass, composed of blood vessels, smooth muscles and mature adipose tissues. These were not epithelioid cells and immunohistochemical stain for human melanoma black (HMB)-45 was negative. Extrarenal AML usually is not associated with tuberous sclerosis and has no HMB-45 immunoreactive cells although it shares the terminology with renal AML. It is regarded as benign. Surgical excision is considered curative.