Abstract
The combination of schistosomiasis and pulmonary hypertension (PH) was always recognized as a very rare one; in medical literature, PH is considered as a manifestation of hepatosplenic schistosomiasis but not a manifestation of schistosomal infection until recently. Only 18.5% of patients that have a documented hepatosplenic schistosomiasis were found with PH. Schistosomiasis rarely causes PH without evident hepatosplenic manifestations. Here, we are reporting a case of a patient whose first clinical presentation was features of PH. We use this case as an opportunity to outline pathological mechanisms, causes and classification of PH. A structured and thorough workup for PH is emphasized. It is important to exclude all other secondary causes to be able to diagnose primary PH especially in the absence of a positive family history and advanced diagnostic technology.