Bone sarcomas are rare tumors arising in bone, representing only a small fraction of solid malignant tumors. Desmoids are benign, infiltrative soft tissue neoplasms. Because of their scarcity and a paucity of data, the management of these tumors can be challenging, especially for clinicians who infrequently encounter these tumors. This article reviews the current literature regarding the diagnosis, work-up, and treatment of these uncommon mesenchymal tumors.