Herlyn-Werner-Wunderlich syndrome, also known as OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly), is an extremely rare abnormality of the Müllerian ducts, characterized by the presence of double uterus, unilateral cervico-vaginal obstruction and renal agenesis and/or other urinary tract anomalies. Most cases are diagnosed after menarche through an MRI, CT or ultrasound. There are no specific symptoms of this syndrome. Patients presented fever and vomiting during menstruation, intermenstrual bleeding and palpable abdominal, pelvic or vaginal mass. Thorough history taking is extremely important as common symptoms in pediatrics (vomiting, constipation, abdominal pain) may be a part of a more complex condition, not only within the GI tract but also including other systems.