Developmental spinal canal stenosis at atlas level is a series of symptoms caused by primary or secondary stenosis of the sagittal diameter of the atlas resulting in compression of the spinal cord [1-2] due to the hypoplasia or deformity of the atlas. The developmental abnormality of the atlas bone structure is the main reason, and its development mechanism is complex, which is related to embryo development. Congenital spinal stenosis is a common risk factor of cervical spondylosis, which is most common between C3-5[3]. It has been widely studied in the lower cervical spine while still lacking at the C1 plane. Dysplasia of the atlas may be a risk factor for developmental stenosis of the atlas. For instability and dislocation of the atlas, the narrow atlas will lead to more severe spinal cord compression. Due to the special anatomical structure, the diagnosis of developmental atlantoaxial stenosis is also specific. The diagnosis value of the stenosis is controversial. ADI in patients with developmental atlantoaxial stenosis with spinal cord compression may also be in the normal range in the conventional sense (male < 4mm, female < 3.5mm). At present, there is no unified standard for the diagnosis, classification and treatment of this disease, and clinical studies are mostly case reports. Therefore, this paper reviews the diagnosis and different treatment strategies of developmental atlantospinal stenosis.