Parasites of the genus Orientobilharzia belong to Platyhelminthes, Trematoda, Digenea, Schistosomatidae, and the type species is Orientobilharzia turkestanicum. O. turkestanicum was first described by Skrjabin from cattle in Russian Turkestan in 1913. Adult worms of Orientobilharzia species live in the portal veins or intestinal veins of cattle, sheep and other mammals, and often cause orientobilharziasis in China, India, Mongolia, Pakistan, Iraq, Iran in Asia, and Russia and Turkey in Europe. More importantly, the cercariae of Orientobilharzia species can infect humans and often cause cercarial dermatitis. Though Orientobilharzia species have been confirmed as zoonotic agents, they have been largely neglected, compared with other pathogens causing cercarial dermatitis, such as Trichobilharzia spp., Schistosoma spindale and Bilharziella sp., which have attracted considerable attention. Here we review the current status of knowledge on the taxonomy of Orientobilharzia spp., human and animal infections with Orientobilharzia spp., and address some considerations for further work on the systematics and pathogenesis of these organisms.