Skull base osteomyelitis is a very rare disease that affects the bone marrow of the temporal bone, sphenoid bone, occipital bone. It occurs typically chronic ear canal infections by malignant otitis externa, but some of atypical osteomyelitis have been reported. It most commonly presents old diabetic patients, and have high morbidity and mortality rate if diagnosis and treatment are delayed. However with respect to pain or dysfunction, it appeared similar to the initial symptoms of temporomandibular joint disorder. So frequently, definitive diagnosis is tend to delayed. We have clinical experience that a patient who presented with symptom similar to temporomandibular disorder, and differential diagnosised by skull base osteomyelitis. We will report this case with literature review.