Chondroid syringoma is a relatively rare benign skin appendageal tumor with an incidence of <0.098%. Malignant chondroid syringoma (MCS) arises from cutaneous sweat glands and occurs on the extremities or trunk more commonly in women with only 51 reported cases. Due to the rarity of the disease and lack of published cases of MCS, the diagnostic criteria and treatment protocols are not clear. Based on available recommendations and histological criteria, MCS was diagnosed in a previously classified elbow lipoma following increased size and pain, and skin color changes in a 65-year-old woman.