Schistosomiasis is one of the most prevalent chronic infectious diseases in the world. One of its most severe complications, pulmonary hypertension, occurs in up to 5% of patients with hepatosplenic schistosomiasis. The prevalence of schistosomiasis is so overwhelming that schistosomiasis-associated pulmonary hypertension (Sch-PH) may be the most prevalent cause of pulmonary hypertension around the world. Multiple pathways have been described as potential mechanisms of disease in Sch-PH, such as egg embolism, inflammatory disease or pulmonary blood overflow. The possible physiopathological mechanisms will be discussed in this article, as well the disease's clinical course and response to the treatment available.