We present the first case report of an endoscopic removal of a living worm, species Dirofilaria repens, from the orbital cavity. As of today, over 410 cases of Dirofilaria repens infections in man are recorded in world literature, six of which were localized in the orbital cavity. In Austria we know of four cases of an infection with this parasite, but none in the orbit. Dirofilaria repens is widespread only in the Old World, particularly in Southern and Eastern Europe, in Asia Minor, and in Central and Southern Asia. The highest prevalence of the disease is recorded in Italy (181 cases). In clinical practice, the infections have mostly been misdiagnosed as a neoplasia, usually benign but sometimes malignant. Under the assumption of an intraorbital tumor, the endoscopic transnasal revision of the orbital cavity was performed, as this approach promised to be least traumatic and best suited for the lesion, resulting in complete removal of the live worm. In unclear lesions in the head and neck, and infection with Dirofilaria repens should be considered as a differential diagnosis.