Pigmented villonodular synovitis (PVNS) is a proliferative disorder of the synovium with a predisposition for the appendicular skeleton. Rarely PVNS can arise from the spine, where this disorder usually presents with localized or radicular pain secondary to involvement of the posterior elements. The authors report the case of an 82-year-old woman who presented with long-standing neck pain and acute upper-extremity numbness and weakness. Computed tomography imaging revealed a mixed sclerotic and lucent lesion affecting the dens and right lateral mass of C-2. There was also a pathological fracture at the base of the dens with 8 mm of anterior dens displacement. Magnetic resonance imaging demonstrated a diffusely infiltrative process that was nonenhancing. Because of instability, the patient underwent transarticular screw fixation, and a biopsy of the lesion was also performed at this time. Histopathological analysis was consistent with a diagnosis of PVNS. To the authors' knowledge, this is the first report of PVNS involving the C-2 vertebra or causing a pathological fracture.