We present a case of a septate uterus with duplication of the cervix and a vaginal septum, which challenges the embryologic concept of unidirectional caudad to cephalad müllerian ducts fusion. This unique case is, to the best of our knowledge, the seventh reported case of this müllerian anomaly. It challenges the classic theory of unidirectional caudad to cephalad fusion of the müllerian ducts, and might be additional evidence to support the hypothesis suggested by Musset et al. Accurate diagnosis and reports of such cases are important not only for the benefit of treatment, but to reflect the true incidence of this anomaly, and consolidate the embryologic concept.

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