The authors report the case of a 66-year-old man with progressive, nontraumatic, C-1 cord compression who presented with a complete but hypoplastic atlas. They review six cases found in the literature. Symptoms usually develop in the late adulthood; the sagittal diameter of the canal measures 10 mm or less. The treatment requires a posterior decompression. Opening of the dura is sometimes necessary. There is no pressing need for a primary stabilization device.