Surgical management and outcome of pheochromocytoma with invasion of the inferior vena cava (IVC) has not been thoroughly reported or widely available in the literature. As determining the malignancy of pheochromocytoma has yet to be clearly defined and the experience of surgical resection of this condition is underrepresented, we include a case report and a systematic literature review on this condition. A literature search in PubMed was performed based on a recent review article by Kassahun et al. (2016). The search yielded 13 case reports between 1988 and 2014 from which data was extracted based on the following variables: patient’s age, gender, comorbidities, laboratory tests for pheochromocytoma diagnosis, size and laterality of the tumor, diagnostic test for IVC invasion, preoperative treatment and imaging, perioperative procedures, and oncological outcomes. The mean age of the patients was 49.4 years, with 38.5% being female (n=5) and 53.3% being male (n=8). 92% (n=12) of the cases were right-sided pheochromocytomas that invaded the IVC, with only one case of pheochromocytoma localized in the organ of Zuckerkandl. Only 46.7% (n=7) of the cases reported using laboratory tests to diagnose pheochromocytoma while only 53.3% (n=8) specifically reported using imaging techniques (CT, MRI, ultrasound, CTA, inferior venacavogram, MIBG-scintigraphy) to diagnose IVC invasion. Four of the surgical cases were performed without cardiopulmonary bypass while three specifically mentioned the use of the bypass technique. There was also further variance in surgical approach as some were performed via sternotomy, thoracophrenolaparotomy, or thoracoabdominal approach to completely resect the tumor and IVC, or to perform a cavotomy with no reconstruction of the IVC necessary. Follow-up of only 8 patients were noted, all of which revealed no evidence of disease, although at different time points post-op. The lack of a thorough and consistent report on pheochromocytoma cases with IVC invasion makes it difficult to compare the management of this condition. We hope a comprehensive review of these patient cases provides greater insight into the diagnosis of pheochromocytoma with IVC invasion and the characteristics and surgical procedure of this tumor invasion that would eliminate recurrence.