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Undescended ovary is a rare congenital gynecologic condition, frequently associated with urogenital malformations including unicornuate uterus and ectopic kidneys or renal agenesis. Although ectopic ovaries are mostly found during an infertility work up, its role in infertility is still unknown. We report a 38-year-old patient presenting with pain in the right lower quadrant. Explorative laparoscopy reveals a unicornuate uterus and a malpositioning of the right ovary and distal ending of the right fallopian tube. Through this report, we try to provide hints for guiding the diagnostic management of similar patients in terms of fertility, renal function and tumour formation.