A 22 year-old man was admitted to Chonnam University Hospital with chief complints of hearing loss, mucoid aural discharge, preauricular pain of right ear and pain on both hips and knee joint as well as disturbance of gait. On physical examination large irregular punched-out defect on the frontal region of skull. Eyeball was slightly prominent. Posterior wall of right external auditory canal revealed marked tissue defect, on which was lined with pinkish granulomatous masses with scanty amount of mucoid secretion. Lower two third of right ear drum was also covered with above mentioned granulomatous mass but no perforation was identified. X-ray film revealed multiple punched-out defect on skull including right temporal bone. Histopathological finding of granulomatous mass taken from right external auditory canal disclosed infiltration of histiocytes with storage of lipids. Pure tone audiometry revealed complete hearing loss of right ear indicating dead labyrinth. Caloric test revealed no response on the right ear. The condition was considerably improved by administration of heavy doses of steorid.