Odontoid osteomyelitis is a rare disease which is frequently misdiagnosed as torticollis, leading to a delay in diagnosis. We present 2 illustrative cases and a systematic literature review summarizing previously reported cases. Odontoid osteomyelitis should be considered in children presenting with decreased head movements and with elevated erythrocyte sedimentation rate, particularly without improvement while receiving antiinflammatory treatment. Plain radiographs can be misleading, and magnetic resonance imaging should be performed for better visualization.