OBJECTIVE: The aim of the study was to report 2 cases of extramedullary plasmacytoma (EP) with localization to middle ear that were diagnosed and managed in our department.METHODS: The first patient was a 60-year-old man with progressive hearing loss, tinnitus, recurrent episodes of otalgia, and otorrhea in his right ear during the last 2 years. The second patient was a 66-year-old man who presented with aural fullness in his right ear and a mild pain in the region of right mastoid. Mild hearing loss and episodes of dizziness with sensation of falling for about 6 months were reported in the clinical history.CONCLUSIONS: The prognosis of EP is considered favorable in regard to the solitary bone plasmacytoma and multiple myeloma (MM). The differential diagnosis of EPs from other plasma cell dyscrasias and especially from MM is considered essential. It is a radiosensitive tumor and, especially for EPs of temporal bone, the combined treatment of surgery resection and postoperative radiation seems to provide the best local control and the lower risk of occurrence. The therapeutic strategy includes a close follow-up of the patients because of the risk of occurrence and/or dissemination into MM. The 10-year survival rate reaches 70% with the appropriate therapy.