Herlyn-Werner-Wunderlich (HWW) syndrome is part of a spectrum of Müllerian duct anomalies that occur during embryonic development. The syndrome is characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Only few cases of this disease were reported worldwide. We present a 23-year-old female patient with chronic pelvic pain for years. The patient was diagnosed with HWW syndrome with a history of hematocolpos and vaginoplasty at the age of 12. Five months later, she sought urgent medical care due to intense pain, and a clinical picture suggestive of peritoneal irritation. Clinical condition deteriorated and the patient underwent an exploratory laparotomy, which failed to identify anything to justify the abdominal pain. Uroculture and blood culture were negative. A magnetic resonance imaging showed a tube-shaped formations extending from the right retrovesical region to the mesogastrium corresponding to the persistence of the mesonephric duct, related to ipsilateral renal agenesis. The patient underwent laparoscopic procedure, identifying a right ureteric remnant blind ending, with distal and proximal obliteration, filled with purulent secretion, which was totally resected. This case differs from the other reported cases due to pelvic pain secondary to infection and abscess in ureteric remnant. We did not find any case with similar clinical presentation.

Herlyn-Werner-Wunderlich (HWW) syndrome is part of a spectrum of Müllerian duct anomalies that occur during embryonic development. The syndrome is characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Only few cases of this disease were reported worldwide. We p...