Background: The majority of primary heart tumors are benign, but they are uncommon. The two most prevalent forms of primary cardiac tumors in adults are cardiac myxomas and papillary fibroelastomas (PFEs). A benign neoplasm is PFE. According to a single series of several patients by some authors, PFEs are the most prevalent kind of cardiac malignancy. The detection of intracardiac malignancies has resulted from the use of sophisticated imaging methods like transesophageal echocardiography (TEE). Objective: This review article aimed to highlight key points and rare presentations of cardiac fibroelastoma in pediatric. Methods: The terms PFE, pediatric, stroke, and cardiac tumor were used to search PubMed, Science direct and Google scholar. Additionally, the writers culled references from the pertinent literature, identifying and included just the most current or comprehensive study out of all the found studies and reviews. The search for literature was limited to English language works. Dissertations, oral presentations, conference papers, unpublished articles, and abstracts from smaller scientific investigations were excluded. Conclusion: Among benign cardiac tumors, cardiac PFE (CPFE) are extremely rare in children. Although most CPFE do not cause symptoms, there is sufficient evidence to state that these tumors can cause life-threatening complications. Therefore, the diagnosis of CPFE in infants and children is of vital importance due to the high risk for embolization. Accordingly, prophylactic tumor excision with valve repair or replacement when necessary is considered to be the treatment of choice, even in asymptomatic patients.