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Five cases of uterus didelphys, unilateral imperforate vagina and ipsilateral renal agenesis are reported
with a follow-up from 1 to 5 years. Diagnosis included typical revelation at puberty (2 cases) but also in the first
months of life (2 cases). One case was only diagnosed at the age of 6 years, and the presence of an ectopic ureter was
noted in the imperforate vagina. The authors point out the necessity of precise evaluation and careful management in
order to maintain an intact reproductory tract. Simple excision of the septum is recommended which can most of the
time be done by the vaginal approach.