Background: rectal atresia is a rare variant of anorectal malformations. The presence of fistulous communication between the rectum and the urogenital system in case of rectal atresia is even rarer to occur. Aim of the work: this article aimed to describe a case of rectal atresia in a female infant associated with proximal and distal congenital rectovaginal fistula. Results: the clinical, radiological findings and the surgical management were discussed . Conclusion: the case demonstrated the possibility to find a rectovaginal fistula in rectal atresia, and the possibility of recurrence after surgical intervention.