Skull base osteomyelitis arises as a complication of malignant external otitis, but it can be also due to middle ear and/or mastoid infection. Other causes can be infections of the paranasal sinuses or of the mandible or maxilla due to odontic caries. Generally, osteomyelitis involves elderly patients affected by diabetic immunodeficiency or microvascular disease. In this paper, we present 3 new cases of skull base osteomyelitis with atypical onset and evolution. The difficulties of diagnosis and details of the management are discussed.