In the treatment of primary tumors, complete local eradication is the main goal, as an oncologically appropriate surgical treatment can substantially improve the prognosis and even be considered a life-saving procedure. In deciding the best treatment for primary bone tumors of the spine, the choice of surgery, radiation therapy, chemotherapy, selective arterial embolization, or other medical treatments alone or in combination is based on diagnosis, staging, and a deep understanding of the biology and the behavior of each tumor. This article is a guide to diagnosing and treating such rare tumors.