Dirofilariosis caused by Dirofilaria (Nochtiella) repens is recorded sporadically among people in Europe, Asia and Africa. Still a worldwide controversy exist upon human parasite hosting. Herein, the first case of ocular dirofilariosis in Bulgaria caused by gravid female is presented. A single nematode was removed from the eye of the 76- year-old patient in the course of cataract surgery. Microscopic examination of the histological slides revealed microfilariae in the parasite’s uterus, but not in the blood. Knott’s method for detection of microfilariae in the peripheral blood was negative and the rest of laboratory blood and biochemical tests were within reference limits. A comprehensive review of the etiology, pathogenesis and clinical presentation of Dirofilaria repens infection is also highlighted.