RESUMO
Obstructed hemivagina and ipsilateral renal agenesis/anomaly (OHVIRA) syndrome is a very rare condition affecting girls. The time of diagnosis varies, from cases of prenatal diagnosis up to adulthood, including pregnancy or delivery. Most commonly, it is recognised during puberty and usually as an acute condition. We present a case report of an adolescent girl who underwent the treatment because of acute abdominal pain. The case is interesting due to a previous diagnosis of one-side renal agenesis. It appears to be useful to perform a diagnostic pelvic imaging at the time of diagnosis of renal agenesis or to plan to perform it at the beginning of puberty, to prevent the need for urgent surgery. This management may allow the planning of proper follow-up, minimising the risk of possible complications.