Pleomorphic adenoma with lipomatous metaplasia is a rare type of pleomorphic adenoma. This paper reports a case of pleomorphic adenoma with lipogenesis. A 28-year-old female patient presented with a mass in front of the left ear for more than 2 years, accompanied by pain. Computed tomography and B-ultrasound examinations suggest the possibility of pleomorphic adenoma. Postoperative pathological results revealed a pleomorphic adenoma with lipomatous metaplasia, and fat accounted for 60% of the tumor. Basing on the literature, we discussed the naming of this tumor and systematically summarized its imaging manifestations, clinicopathological features, differential diagnosis, and treatment.
