Metastatic pheochromocytoma of the spine (MPS) represents an extremely rare and challenging entity. While retrospective studies and case series make the body of the current literature and case reports, no systematic reviews have been conducted so far. This systematic review aims to perform a systematic review of the literature on this topic to clarify the status of the art regarding the surgical management of MPS. A systematic review according to PRISMA criteria has been performed, including all studies written in English and involving human participants. 15 papers for a total of 44 patients were finally included in the analysis. The median follow-up was 26.6 months. The most common localization was the thoracic spine (54%). In 30 out of 44 patients (68%), preoperative medications were administered. Open surgery was performed as the first step in 37 cases (84%). Neoadjuvant treatments, including preoperative embolization were reported in 18 (41%) cases, while adjuvant treatments were administered in 23 (52%) patients. Among those patients who underwent primary aggressive tumor removal and instrumentation, 16 out of 25 patients (64%) showed stable disease with no progression at the final follow-up. However, the outcome was not reported in 14 patients. Gross total resection of the tumor and spinal reconstruction appear to offer good long-term outcomes in selected patients. Preoperative alpha-blockers and embolization appear to be useful to enhance hemodynamic stability, avoiding potential detrimental complications.