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Acute promyelocytic leukemia (APL) is a unique subtype of acute myelogenous leukemia for which complete remission is induced by using anticancer drugs with all-trans retinoic acid (ATRA). Extramedullary involvement at relapse is occasionally observed in patients with APL, and is said to be more common after treatment with ATRA. We describe a 35-year-old man who had a relapse due to a mass that developed in the external auditory canal after treatment with ATRA. He was successfully treated with arsenic trioxide and radiotherapy. Korean J Otorhinolaryngol-Head Neck Surg 2016;59(9):688-91