Schistosomiasis japonica is a serious parasitic disease and a major health risk for more than 60 million people living in the tropical and subtropical zones of south China. The disease is a zoonosis and its cause, the parasitic trematode Schistosoma japonicum, has a range of mammalian reservoirs, making control efforts difficult. Current control programs are heavily based on community chemotherapy with a single dose of the highly effective drug praziquantel. However, vaccines (for use in bovines and in humans) in combination with other control strategies are needed to eliminate the disease. In this review, we provide an overview of the transmission, clinical features, pathogenesis, diagnosis, treatment, genetics and susceptibility, epidemiology, and prospects for control of schistosomiasis japonica in China. The threat posed by the Three Gorges Dam may undermine control efforts because it will change the local ecology and associated schistosomiasis transmission risks over the next decade and beyond.