Introduction: OHVIRA syndrome is a rare entity characterized by renal and Mullerian anomalies. The objective of the video is, through a clinical case, to discuss the importance of diagnosis, management and treatment, to avoid the complications that this syndrome entails, and to improve the long-term prognosis.
Materials and methods: We report the case of a 10-year-old girl who consulted for abdominal pain, being diagnosed with OHVIRA syndrome. We describe the diagnosis and the surgical technique. In addition, we perform a systematic review in PubMed to report the published literature of this topic and we show the optimal management of this pathology.
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