Lateral atlantoaxial osteoarthritis (AAOA), or C1-C2 lateral mass arthritis (LMA), is an unfamiliar degenerative cervical disease with a clinical presentation that markedly differs from subaxial spondylosis. The prevalence of LMA in the nonsurgical outpatient setting is 4%. Risk factors include age and occupation. The typical patient is between 50 and 90 years old, presents with upper cervical or occipital pain, has limited rotation, and has pain provocation during passive rotation to the affected side. Pain stems from degeneration of the lateral C1-C2 articulation and may be referred or radicular, through the greater occipital nerve. Although there is no consensus on diagnostic work-up, the disease is classically seen on the open-mouth odontoid radiograph. Computerized tomography, magnetic resonance imaging, bone scan, and diagnostic injections are also useful. Initial treatment is conservative, and upwards of two-thirds of LMA patients obtain lasting relief with noninvasive measures and injections. In patients with severe, recalcitrant pain, limited C1-C2 fusion offers satisfactory and reliable relief. The goals of this review article are to provide a synthesis of the literature on LMA, to offer a treatment approach to LMA, and to identify problems with the current state of knowledge on LMA.