In a retrospective review of seven patients with AIDS who were diagnosed with necrotizing external otitis between 1990 and 1995, it was found that the presentation of necrotizing external otitis in patients with AIDS differed from the classic description of malignant external otitis in several respects. The patient population was significantly younger and nondiabetic. Granulation tissue was usually absent from the external auditory canal and Pseudomonas aeruginosa was not the predominant pathologic organism. Also, outcome was found to be significantly worse. Thus a high index of suspicion must be entertained and vigorous local and systemic treatment initiated early in the course of disease to achieve a satisfactory outcome.