The developmental anomalies of the reproductive system are frequently accompanied by urinary system anomalies. Among the development anomalies of the urogenital system, the syndrome of double uterus with occluded one vagina and lack of kidney on the same side is very rarely encountered and, in view of this, little known. This anomaly may be frequently accompanied by other developmental anomalies. The authors presented such a case. Thanks to the theoretical knowledge its diagnosis and adequate management were facilitated.
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