The occipitocervical junction (OCJ) is a highly specialized area of the spine. Understanding the unique anatomy, imaging, and craniometry of this area is paramount in recognizing and managing the potentially devastating effects that pathology has on it. Instrumentation techniques continue to evolve, the goal being to safely obtain durable, rigid constructs that allow immediate stability, anatomical alignment, and osseous fusion. This article reviews the pathologic conditions at the OCJ and the current instrumentation and fusion options available for treatment. The general orthopedist needs to recognize the pathology common in this region and appropriately refer patients for treatment.