| Summary: | Abstract Background Müllerian anomaly is a congenital defect in the development of the female reproductive system that varies according to the stage of developmental failure resulting in agenesis, hypoplasia, or fusion defect in one or both Mullerian ducts; in such patients, it is expected to have higher pregnancy and delivery complications. Case presentation This case presentation of a 14-year-old adolescent girl with uterine didelphys, obstructed right hemi-vagina, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome, HWW) that was corrected surgically, then got married at the age of 16 with successful two consecutive term uncomplicated pregnancies of favorable maternal and fetal outcomes without a history of any miscarriage or recurrent collections. Conclusions Herlyn-Werner-Wunderlich syndrome is a rare case and represents a challenge to diagnose and treat especially in pediatrics and adolescents. Early recognition and treatment will help to improve outcome and reduce possible complications resulting from obstruction with retrograde menstruation and its consequences. Successful early management of our patient improved her quality of life by relieving her cyclical obstructive pain, preserve fertility, and normal sexual life.
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