Combination fractures of the atlas and axis occur relatively frequently and are associated with an increased incidence of neurological deficit compared with either isolated C1 or isolated C2 fractures. C1-type II odontoid combination fractures are the most commonC1-C2 combination fracture injury pattern, followed by C1-miscellaneous axis body fractures, C1-type III odontoid fractures, and C1-Hangman combination fractures. Class III medical evidence addressing the management of patients with acute traumatic combination atlas and axis fractures describes a variety of treatment strategies for these unique fracture injuries based primarily on the specific characteristics of the axis fracture injury subtype. The type of axis fracture present generally dictates the management strategy for the C1-C2 combination fracture injury. Rigid external immobilization is typically recommended as the initial management for the majority of patients with these injuries. Combination atlas-axis fractures with an atlantoaxial interval of ≥5 mm or angulation of C2 on C3 of ≥11° have been considered for and successfully treated with surgical stabilization and fusion. Surgical options in the treatment of combination C1-C2 fractures include posterior C1-2 internal fixation and fusion or combination anterior odontoid and C1-2 transarticular screw fixation with fusion. Fractures of the posterior ring of the atlas can complicate the surgical treatment of unstable C1-C2 combination fracture injuries. If the posterior arch of C1 is incompetent and a dorsal operative procedure is indicated, occipitocervical internal fixation and fusion, posterior C1-C2 transarticular screw fixation and fusion, and C1 lateral mass-C2 pars/pedicle screw fixation and fusion techniques have been reported to be successful. © 2013 by the Congress of Neurological Surgeons.