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BACKGROUND: Pneumocystis carinii (PC) otitis is a rare opportunistic infection of the acquired immunodeficiency syndrome (AIDS). Initially hearing loss and otalgia occur with thickening of the tympanic membrane and the bordering skin of the ear canal. Otorrhea, ear polyps, perforation of tympanic membrane, destruction of mastoidal bone, and participation of cranial nerves are observed. Diagnosis is established histologically. The treatment of the parasite is by trimethoprim-sulfamethoxazole combinations. The immunological situation seems to be better than in PC pneumonia. Due to the underlying immunological incompetence the infection can not be expected to limit itself. To prevent severe complications as sequestrating mastoiditis, early diagnosis and specific surgical and medical treatment are necessary.