Atlantoaxial kyphosis (AAK) is a rare sagittal deformity of the occiptoatlantoaxial junction. It is defined as a subgroup of anterior translatory atlantoaxial instability. AAK is a symptom of several ligamentours or bony disorders of the craniocervical junction; however, rheumatoid arthritis and trauma are the most common causes for AAK. AAK can be diagnosed on lateral radiographic views of the upper cervical spine if the angle between McGregor's line and the atlas plane is less than-15 degrees or the atlas-axis angle is greater 105 degrees. Treatment modalities for AAK depend on the ability to reduce the deformity. If closed reduction is achieved, posterior atlantoaxial fusion by sublaminar wiring according to Brooks or transarticular screw fixation according to Magerl are possible choices. Irreducible AAK can be treated with a combined transoral decompression, anterior plating according to Harms, and posterior wiring according to Brooks. This staged therapy for AAK was successful in our rheumatoid patient population with AAK.