OHVIRA syndrome is a rare Müllerian ductal anomaly with uterine didelphysis, hemivaginal obstruction, and ipsilateral renal agenesis that is usually diagnosed in adolescence, but rarely later. The presented case concerns a 49-year-old woman with severe dysmenorrhea and intermittent spotting. MRI confirmed the diagnosis: didelphic uterus with hematometra and hematocolpos on the right due to hemivaginal obstruction and absence of the right kidney. Treatment consisted of vaginoscopic and hysteroscopic resection of the vaginal septum. After the operation, the symptoms completely resolved and there was no recurrence at the 6-month follow-up. The case documents one of the latest known presentations of the syndrome and emphasizes the need to suspect Müllerian anomalies at any age. A partial drainage hole can mask symptoms for decades, and a minimally invasive surgical approach is effective in the long term.