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Skull base osteomyelitis (SBO) is classically described as an infection of the external auditory canal invading the base of the skull. SBO occurs most commonly in elderly diabetics and is almost uniformly caused by Pseudomonas aeroginosa. Fungal SBO is very rare and usually caused by Aspergillus, and less commonly by Candida. The diagnosis of fungal SBO can be delayed because cultures growing fungal pathogens are slow and often interpreted incorrectly as an opportunistic colonization. This may lead to delayed treatment and greater morbidity. The lack of definitive bacterial cultures and inadequate responses to antipseudomonal therapy should raise the possibility of fungal infection, and biopsy of skull base should be obtained to confirm the causative organism, as well as ruling out an underlying malignancy. Fungal SBO is a medical, not a surgical disease. Surgery may also be indicated if the disease plateaus or doesnt on appropriate antimicrobial therapy. We have recently encountered two rare cases of SBO that were caused by Candida. The patients were much improved following mastoidectomy and intravenous amphotericin B. (Korean J Otolaryngol 2006;49:743-6)