Objective: 1) Present an abnormal case presentation of an already rare and deadly disease process. 2) Critically review/summarize the literature as it pertained to this case. 3) Increase awareness of a diagnostically challenging reversible deadly disease process. 4) Review current treatment options/recommendations for atypical SBO. Method: This is an unusual case of atypical skull base osteomyelitis in an immunocompetent patient diagnosed in 2010 at the Cleveland Clinic Foundation (CCF). A literature review for “atypical” and “central skull base osteomyelitis” was performed using PubMed. Results: As with other published cases of atypical skull base osteomyelitis, this patient was an elderly male, had no preceding infective source, and presented initially with headache that progressed to multiple lower cranial neuropathies (CN 8, 9, 10, 12). However, our patient is unique because he was not diabetic and had no immunodeficiencies. His work-up initially centered on ruling out a primary headache disorder, temporal arteritis, and oncologic pathology. Biopsy, culture, and an indium tagged WBC scan were pivotal in proving an infectious etiology. Prolonged IV antibiotics were vital in clearing the infection. Conclusion: Skull base osteomyelitis should be considered even in immunocompetent patients presenting with headache and cranial neuropathies. When possible, culture, biopsy, and indium-tagged-WBC scan should be performed to help distinguish an infectious process over malignancy, and guide antibiotic therapy. Early diagnosis and treatment is imperative in managing this life-threatening disease.