Atlanto-axial instability (AAI) is a developmental anomaly often occurring in persons with Down's syndrome (DS). According to various reports, AAI affects from 6.8 to 27% of the population with DS. The aim of this review was to illustrate the issue of AAI with regard to the progressively changing state of scientific knowledge. The extended distance between the rear surface of the frontal arcus of the C1 cervical vertebra and the anterior surface of C2 cervical vertebra dens (anterior atlanto-odontoid distance, AAOD) indicates the occurrence of AAI and is detectable through Xray examination. Hypoplasia of the C2 dens, also detectable through X-ray examination, is another suggested risk factor for AAI. According to current data, the methodology of taking measurements is inconsistent, which leads to errors in interpretation. As research focusing on AAI was progressing, new data emerged from other studies on persons with DS, suggesting that neurological symptoms in persons with DS that indicated the occurrence of spinal cord compression were an important factor in medical imaging detection of AAI. One of the main arguments supporting this thesis is that in isolated cases