Dioctophyme renale has a worldwide distribution and has been diagnosed in several wild and domestic animals as well as in humans. As numerous reports in the literature exist concerning the presence of D. renale in different animal species, as well as its diagnosis, treatment, and confirmation in new geographic areas, we reviewed existing information to contribute to the knowledge of the etiology, biology, epidemiology, pathogenesis, symptoms, diagnosis, treatment, and prevention of D. renale. Results of dioctophymosis may range from asymptomatic infection to even death of the host. Diagnosis is based on data from morphology, imaging, and antibody testing, with surgical treatment being the most effective. A high potential for infection of pets exists when there is overlap with wild parasitized animals; given common risk factors for infections in humans, D. renale should be considered as having zoonotic potential.