Inflammatory pseudotumor is a pathologically benign condition, but it demonstrates a wide range of clinical features ranging from silent small sized tumors to aggressive features mimicking malignancy. Pseudotumors most commonly occur in the orbital area, and the involvement of the middle ear cavity is extremely rare. Several modalities are known for the treatment of pseudotumors, including complete surgical excision, oral steroid therapy, and radiation therapy. We describe a 35-year-old woman with inflammatory pseudotumor involving the middle ear cavity. The patient was treated with canal wall up tympanomastoidectomy and additional treatments with steroid and radiation therapy. However, she showed side effects to high dose steroid treatment and no response to radiation therapy. Therefore, we decided to use methotrexate with low dose steroid. After treatment, symptoms were completely resolved and there was no evidence of recurrence 1 year after maintaining immunosuppressant treatment. � Korean J Otorhinolaryngol-Head Neck Surg 2013;56:532-4