To clarify etiology, clinical features, and diagnostic and treatment options of atlantoaxial dislocation (AAD) due to os odontoideum (OsO) in patients with Down's syndrome (DS).We described and analyzed three clinical cases of AAD due to OsO in DS patients and reviewed descriptions of similar cases in the scientific sources.According to literature review, more than 80% of DS patients with odontoid ossicles had atlantoaxial instability (AAI). AAI in DS patients with OsO is more often manifested in childhood and adolescence, rarely in adults when ligament relaxation is reduced. Some patients had acute clinical manifestation after a minor trauma without any precursors; in some of the cases, neurological deterioration increased during several years. We found that the earlier surgical treatment of AAD due to OsO in DS patients carries the higher recovery potential.Most patients with DS and OsO had AAI. The method of appropriate treatment in such cases is a posterior screw fixation. Preoperative halo traction and posterior fusion have proved to be a very useful tool in the treatment of AAD due to OsO in DS patients. Even if irreducibility of the AAD established preoperatively, it should not be an absolute indication for anterior decompression. In such cases, an attempt to reduce the AAD should be made under general anesthesia during posterior fixation.