Fetus in fetu (FIF) is a rare congenital anomaly that occurs when a malformed parasitic twin grows within the host twin (most frequently in the retroperitoneum). FIF is a rare condition, with a high diagnostic and surgical challenge for neonate and infant patients due to its similarity to retroperitoneal teratoma. The purpose of this study was to review the clinical presentation, radiological findings, surgical management and outcomes of FIF in neonates and infants by analyzing 80 cases of FIF reported between 2000 and 2025. Pediatric surgery journals, radiology reports, medical databases such as PubMed, Scopus, and Google Scholar, were used to collect data. The results indicated that the abdominal distention and palpable abdominal mass were the most common symptoms and male infants were more frequently affected. CT scan and MRI were very helpful for the identification of vertebral columns, limb buds and calcified skeletal structures, which aided in differentiating FIF from retroperitoneal teratoma. Surgical resection led to good postoperative results, with low recurrence and postoperative complications. All cases were diagnosed by histopathological examination. Prompt diagnosis and surgical intervention are still vital for favorable management and neonatal outcomes.