Sclerosing sweat duct carcinoma (SSDC) is a rare cutaneous neoplasm. A 28-year-old man presented with 2-3 years history of a tetter on his upper lip, and a 3 cm x 4 cm lymph node was palpable in the left submandibular area. MRI showed a mass in the upper lip extending into the buccal mucosa and orbicular muscle, and multiple cervical lymph nodes metastasis on both sides was suspected. A biopsy was performed to have revealed syringomatous carcinoma. The patient underwent operation of extended removal of the upper lip along with right upper neck dissection and left radical neck dissection. 8 cm x 6 cm sized free forearm flap was used for the reconstruction of the lip. Postoperative course of the patient was uneventful and he has no signs of recurrence so far. Pertinent literatures on this rare tumor are reviewed.
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