Unusual variant of anorectal malformation with rectouterine fistula: a rare congenital anomalies, management and review of literatures

Abstract Background Anorectal malformations (ARMs) represent a spectrum of congenital anomalies involving the distal anus, rectum, and urogenital tracts. A rectouterine fistula is an extremely rare variant in females, with limited reports and no standardized management approach, particularly in low-resource settings. Case presentation We report a 7-day-old female neonate with imperforate anus and intestinal obstruction. Clinical evaluation and surgical exploration revealed a high-type ARM with a fistulous connection between the rectum and uterine body, classified according to the Krickenbeck system as a rare variant of a fistula to the female genital tract. The initial management involved laparotomy, fistula ligation, and formation of an end colostomy. The postoperative recovery was uneventful. The patient was scheduled for a definitive pull-through anorectoplasty after 9–12 months, once growth and nutritional status improved. Conclusion This case represents one of the few documented examples of congenital rectouterine fistula in the neonatal period. A staged surgical approach remains safe and effective in resource-limited settings. Early diagnosis, multidisciplinary collaboration, and individualized surgical planning are crucial for optimal outcomes.