A 55-year-old woman was seen in the emergency department with posterior neck pain and a headache after a traffic accident. Physical examination revealed tenderness on palpation over the posterior skull and a midline spinous process of the cervical spine without neurologic deficit. A plain radiograph of the cervical spine demonstrated the absence of the lateral portion of the posterior arch of the atlas and very lucent shadowing of the anterior midline of the atlas, suggesting a fracture of the anterior arch. On three-dimensional computed tomography (CT) of the cervical spine, anterior and posterior bony defects of the atlas were noted. Well-corticated defects were noted with sclerotic change and with no evidence of soft tissue swelling adjacent to the bony discontinuities, consistent with a congenital abnormality. With conservative therapy, the patient gradually showed a lessening of the midline tenderness. Careful investigation with radiography or CT is needed for these patients to avoid confusion with a fracture, because these patients seldom need surgical treatment.