Down syndrome and juvenile rheumatoid arthritis have been associated with ligament laxity, specifically the atlantoaxial ligament, which maintains the proper positioning of the cervical first and second vertebrae. The joint consists of different pathological processes, and it is paramount that individuals with either condition be screened appropriately before surgery is contemplated. The purpose of this paper was to present a case of an individual with both conditions and describe precautionary measures that were undertaken to safely complete dental treatment under general anesthesia and avoid morbidity.