Information on zoonotic Onchocerca species and their insect vectors in Japan is reviewed. At least six Onchocerca species are known in four kinds of ungulates : O. cervicalis in horse (Equus caballus); O. gibsoni, O. gutturosa, O. lienalis and O. sp. (probably same as O. suzukii) in cattle (Bos taurus); O. skrjabini and O. suzukii in Japanese serow (Capricornis crispus); and O. sp. (probably same as, or near to, O. skrjabini) in Japanese deer (Cervus nippon). Five blackfly species serve as natural vectors of three bovine Onchocerca species : Simulium bidentatum for O. gutturosa; S. arakawae, S. daisense and S. kyushuense for O. lienalis; and S. aokii, S. arakawae, S. bidentatum and S. daisense for O. sp. Little is known on the transmission of the other Onchocerca species. Two human cases of zoonotic onchocerciasis found in Japan are also reviewed. O. gutturosa is considered the causative species in both cases, with the possibility of O. sp. (parasite of deer) being involved in the second case.