The persistent vitellointestinal duct is the rarest of all vitelline duct anomalies. This systematic literature review was undertaken to investigate and review the demographics, clinical presentation, surgical procedure performed, and outcome of infants who were operated upon for the persistent vitellointestinal duct. Infants with incomplete desired details, and children/cases operated upon for the above-mentioned anomaly after infancy were excluded. Published literature/manuscripts were retrieved for the previous fifty years from 1971 to December 2021, and the manuscripts were retrieved through an online database search. This review is presented in accordance with the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. A total of n = 280 infants who were operated upon for the patent vitelline duct were recruited for the systematic review. More than three-fourths (n = 217, 77.5%) of infants were boys. Ninety percent of infants were under the age of 3 months, and 72% were neonates/newborn babies. Discharges through the umbilicus (n = 148; 52.85%), and prolapse of the bowel through the umbilicus (n = 117; 41.78%) were the chief complaints in the above-mentioned infants. Operative procedures were performed (n = 280) in the following order of frequency: (1) resection of the persistent vitellointestinal duct, small adjacent segment of ileum, and ileo-ileal anastomosis (n = 121, 43.21%); (2) resection of the persistent vitellointestinal duct, wedge resection of ileum, and ileal repair (n = 88; 31.42%); and (3) other surgical procedures (n = 11, 3.92%). Operative details were not clear/not available/not provided for n = 60 (21.42%) of the infants. Peri-umbilical incisions were more frequently employed for surgical therapy. This review revealed postoperative death in n = 17 (6.07%) infants. The persistent vitellointestinal duct must be surgically resected at the earliest, preferably during the neonatal period.
