Osteoid osteoma is a benign bone tumor, which rarely occurs in the skull, and often causes local pain that is worse at night, characteristically relieved by aspirin. Giant osteoid osteoma in the occipital bone is uncommon. We present a case of a 42-year-old female with an osteoid osteoma of the occipital bone that was totally resected. We discuss the clinical presentation, radiographic findings, and differential diagnosis of occipital osteoid osteoma.