Complete duplication of vagina, cervix and uterus, with imperforate hemivagina and renal agenesis is a rare condition with less than 120 cases published. In those articles, urological complications are uncommon with only a 10% of the total. We report 2 cases of uterus didelphys with unilateral hematocolpos and ipsilateral renal agenesis with urological clinical complications. One of them presented a very rare onset complaining of acute urinary retention. The other patient was referred for difficulty in micturition and abdominal pain which is the most common symptom of this entity. A description of both cases and a literature review of this congenital complex syndrome and its urological complications are reported.

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