Introduction.Uterus didelphys results when Mullerian duct fusion is completely arrested during development. We presented a rare case of nonobstructive uterus didelphys occurring simultaneously with an endometriotic cyst of the ovary. Case report. A twenty-nine-year-old, nulliparous patient was admitted to our Clinic for laparoscopic treatment of an endometriotic ovarian cyst. Diagnoses of right ovarian endometriotic cyst and nonobstructed uterus didelphys were established with bimanual pelvic exam and two-dimensional transvaginal ultrasound. Diagnoses were subsequently confirmed by laparoscopy and magnetic resonance imaging. Laparoscopic incision and drainage of the endometriotic cyst were performed, followed by biopsy and coagulation of endometriotic lesions. Histopathology confirmed ovarian endometriosis. Gonadotropinreleasing hormone analogue (GnRHa) was prescribed postoperatively, for a total of 3 months. Ten months after completion of treatment, the patients was without disease recurence. Conclusion. Nonobstructive uterus didelphys is rarely associated with ovarian endometriosis.Uvod. Dvostruka materica nastaje kada potpuno izostane fuzija Milerovih kanala. U radu je prikazan redak entitet dvostruke materice neopstruktivnog tipa, udružene sa endometriotičnom cistom jajnika. Prikaz bolesnika. Pacijentkinja stara 29 godina, nulipara, primljena je na našu kliniku za laparoskopsku operaciju endometriotične ciste jajnika. Bimanuelnim ginekološkim pregledom i transvaginalnim 2D ultrazvukom dijagnostikovana je endometriotična cista desnog jajnika i dvostruka materica neopstruktivnog tipa. Dijagnoza je kasnije potvrđena laparoskopijom i magnetnom rezonancom. Urađena je laparoskopska incizija i drenaža sadržaja ciste, sa biopsijom i koagulacijom endometriotičnog žarišta. Patohistološki je potvrđena endometrioza jajnika. Postoperativno pacijentkinji je ordiniran analog gonadotropin oslobađajućeg hormona, u vremenu od tri meseca. Deset meseci nakon završenog kompletnog tretmana, pacijentkinja je bila bez recidiva bolesti. Zaključak. Dvostruka materica neopstruktivnog tipa je retko udružena sa endometriozom jajnika.