Synovial chondromatosis is a rare pathology of unknown etiology characterized by multinodular cartilaginous proliferation in the synovium. When it does occur, it usually develops in the large synovial joints (e.g., the knee, hip, elbow, and shoulder). Only rarely does it occur in the head and neck (most often in the temporomandibular joint). We report a case of synovial chondromatosis of the middle ear (i.e., the incudomalleolar joint) in a 64-year-old woman, and we describe the clinical, radiologic, and intraoperative findings in this case. To the best of our knowledge, this is the first reported case of synovial chondromatosis in the middle ear.