This report describes a case of congenital segmental agenesis of the uterine body, cervix and vagina in a bitch. A five-year-old, Pomeranian bitch was presented with anorexia, abdominal distension and absence of the oestrus vulvar discharge. Complementary examinations were performed and ultrasonography revealed marked distention of the uterine horns with fluid-filled thin wall and defects in the uterine-vaginal segment. During surgery, closer inspection of the reproductive tract showed segmental agenesis of the uterine body, cervix and cranial part of vagina. The dog recovered uneventfully and six months later there were no further specific problems.