This study reports the first known case of human hydatid disease caused by the larval stage of Echinococcus oligarthrus. The patient, a native Venezuelan female, presented a single cyst localized intraorbitally behind the left eye (retroocular), which was discovered by computed tomography. The cyst was removed by surgery, and after parasitological studies it was identified as an E. oligarthrus hydatid cyst. This is also the first case of intraorbital hydatid cyst in humans in Venezuela.