Extra-abdominal desmoid tumors are extremely rare in the hand. These tumors do not metastasize; however, they are potentially locally invasive and have extremely high local recurrence rates after surgical excision with reports of up to 78% recurrence, specifically in the hand. We describe the first case of a desmoid tumor originating from the extensor mechanism of a digit and discuss our treatment approach. In addition, a literature review performed shows a male predominance of desmoid tumors in the hand in the 30- to 50-year-old age group. Current evidence supports aggressive early wide surgical excision and reconstruction to preserve function.