We report on a case of pyogenic C1-C2 osteomyelitis that was diagnosed as occipital neuralgia. A 61-year-old man complained of occipital and neck pain with difficulty in rotating his head. Because his abnormal state was not observed and his pain did not diminish, he was referred to our outpatient clinic. His pain was not alleviated by various analgesics, superficial cervical plexus block, or greater occipital nerve block. Therefore we administered hydrocortisone intravenously for the purpose of diagnosing and treating potential systemic diseases, such as collagen disease. After administration of hydrocortisone, his pain disappeared dramatically. We immediately again performed an examination of his neck with X-ray and MRI, and we diagnosed his illness as pyogenic C1-C2 osteomyelitis. The patient received administration of antibiotics to reduce cervical infection and was provided with a neck collar. In conclusion, we should question C1-C2 osteomyelitis if nerve block or administration of analgesics is not effective for unidentified occipital pain.