This paper describes a patient who presented at our hospital with neurologic symptoms due to congenital cervical spinal stenosis at the atlas level. Congenital atlantal stenosis is usually due to hypoplasia of the posterior arch of the atlas. It is a rare cause of spinal stenosis, and only 12 symptomatic patients with isolated atlantal stenosis have been reported. Current treatment is surgical decompression, and all reported patients receiving surgical treatment improved to some degree.