Case: We describe the case of a dysplasia epiphysealis hemimelica (DEH) lesion affecting uniquely the lunate bone in a 16-year-old boy causing pain, stiffness, and decreased range of motion. Surgical excision was performed, leading to resolution of primary symptoms with residual limitations in terminal wrist extension. Conclusions: From our review of the literature, we believe this case to be the first report of a DEH lesion affecting solely the lunate bone. Although conservative and surgical management have both been described with DEH cases, we report a positive outcome following the excision of a symptomatic lesion.