Primary spinal primitive neuroectodermal tumors (PNETs) are uncommon malignancies that are increasingly reported in the literature. Spinal PNETs, like their cranial counterparts, are aggressive tumors and patients with these tumors typically have short survival times despite maximal surgery, chemotherapy, and radiation. Because no standard management guidelines exist for treating these tumors, a multitude of therapeutic strategies have been employed with varying success. In this study the authors perform a comprehensive review of the literature on primary spinal PNETs and provide 2 new cases that highlight the salient features of their clinical management.