Objective: To review the current literature on the state of the art of evaluation and management of cardiac papillary fibroelastoma (PFE). Methods: A literature review was performed on the topic of evaluation and management of PFE. PubWeb, Web of Science, Google Scholar, Scopus search engines were used. Key search terms included: “Cardiac papillary fibroelastoma,” “papillary fibroelastoma,” “Surgical resection of papillary fibroelastoma,” “Minimally invasive resection of papillary fibroelastoma,” “Thoracoscopy and PFE.” Relevant articles were archived and synthesized in an EndNote database. Particular attention was focused on identifying cases of minimally invasive resection of PFE and the use of thoracoscopic adjuncts to assist with cavitary PFE removal. Institutional Review Board approval was waived. Results: A total of 119 articles were identified and archived. Forty seven articles were used to compose this paper. 8 articles detailed minimally invasive resection of PFE. The largest series of minimally invasive PFE resection summed 4 patients. There are currently no guidelines to direct care of patients with PFE. General consensus is that the presence of left sided PFE should prompt evaluation for surgery. Conclusions: PFE is a rare tumor, more common in children than adults. Lesions are typically left sided and are at risk for embolization. Minimally invasive surgery is feasible and safe. Long-term outcomes with complete resection are very good.