Malignant otitis externa (MOE) is an infection of the external auditory canal that invades the skull base. Aspergillus species fungi were the pathological organism in 21 of 23 reported cases of fungal MOE. We report on a 21-year-old man with end-stage acquired immunodeficiency syndrome (AIDS) and fungal MOE caused by Scedosporium apiospermum. Fungal MOE is most common in patients with end-stage AIDS and hematologic malignancies. Granulation tissue is not a common finding in these patients, and the infectious process often starts in the mastoid air cells or middle ear space, as opposed to the external auditory canal. Surgical debridement and amphotericin B are the mainstays of therapy; resolution of the infection depends greatly on the severity of the underlying disease.