The diagnosis of the Herlyn-Werner-Wunderlich syndrome is frequently misleading, not only to underreporting of cases, but, more seriously, to suboptimal treatment that can be detrimental to the reproductive potential of the affected woman. The case of a 14-year old girl with the HWW syndrome is presented. Although the diagnosis was suggested by imaging modalities such as ultrasound and magnetic resonance imaging, the use of endoscopic procedures such as laparoscopy and hysteroscopy, provided additional vital information that allowed an accurate assessment of her condition and a definitive, minimally-invasive management that was protective of her reproductive function.
Human ; Female ; Adolescent ; MULLERIAN DUCTS ; HEREDITARY RENAL AGENESIS

Herlyn-Werner-Wunderlich Syndrome (HWWS) is a triad of uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis.
In the review of the locally published literature, there have been seven HWWS cases reported, none of whom were pregnant.
A 24-year-old was diagnosed with Herlyn-Werner-Wunderlich Syndrome during caesarean section of a term pregnancy, occupying the right hemiuterus with obstructed hemivagina. Ultrasound showed uterus didelphys with communicating endometrial cavities. MRI revealed uterus didelphys, two cervices and an obstructed right hemivagina. The patient refused excision of vaginal septum. Two years later, she delivered spontaneously to a live fetus, occupying the hemiuterus with the unobstructed hemivagina.
In pregnant women with HHWS, who did not undergo prior surgical intervention, the mode of delivery depends on the side of pregnancy. If it is located on the obstructed hemivagina, caesarean section is inevitable. If it is on the unobstructed side, there is hope for vaginal delivery.

Human ; Female ; Adult ; Pregnancy ; Hereditary Renal Agenesis ; Uterine Anomalies ; Uterus ; Urogenital Abnormalities ; Ultrasonography ; Cesarean Section ; Magnetic Resonance Imaging ; Fetus