Combination fractures involving fractures of both the atlas and axis occur relatively frequently. A higher incidence of neurological deficit is associated with C1-C2 combination fractures compared with either C1 or C2 fractures in isolation. The C1-Type II odontoid combination fracture seems to be the most common combination injury subtype, and then C1-miscellaneous axis, C1-Type III odontoid, and C1-hangman's combination fractures. No Class I or Class II evidence addressing the management of patients with combination atlas and axis fractures is available. All of the articles reviewed describe case series or case reports containing Class III evidence supporting a variety of treatment strategies for these unique fracture injuries. In most circumstances, the specifics of the axis fracture will dictate the most appropriate management of the combination fracture injury. As reported for isolated atlas and axis fractures, most atlas-axis combination fractures can be effectively treated with rigid external immobilization. Combination atlas-axis fractures with an atlantoaxial interval of 5 mm or more or angulation of C2-C3 of 11 degrees or more may be considered for surgical fixation and fusion. The integrity of the ring of the atlas must often be taken into account when planning a specific surgical strategy using instrumentation and fusion techniques. If the posterior arch of C1 is inadequate, both incorporation of the occiput into the fusion construct (occipitocervical fusion) and posterior C1-C2 transarticular screw fixation and fusion have been successful.