Atlantoaxial rotatory dislocation usually occurs due to trauma, tumor, or congenital diseases. Diagnosis and treatment are difficult because it happens in the anatomically most challenging and complex region of the craniovertebral junction. Many classifications and treatment methods have been identified in the literature, and efforts have been made to determine the most effective treatment strategy. This article aims to better understand the diagnosis and treatment algorithm by describing the definition, classification, and treatment methods of atlantoaxial rotatory dislocation, thereby reducing the development of complications.