Unilateral ovarian agenesis and fallopian tube maldescent

Unilateral ovarian agenesis (UOA) and fallopian descent problems are very rare congenital defects. We present an unusual case of UOA associated with fallopian-tube maldescent discovered incidentally during a laparotomy in a female infant for a persistent heterogeneous right ovarian cyst refractory to needle aspiration. A necrotic, hemorrhagic paratubal cyst was found associated with a normal right adnexa. The left ovary was absent and a rudimentary left fallopian tube was found tightly stretched over the sigmoid colon to the left retroperitoneum. This previously unreported constellation of anomalies may carry significant potential risks for bowel obstruction and later fertility.