Pulmonary hypertension is a devastating complication of various, but rare diseases and can also occur as an isolated entity. It causes morbidity and mortality in all patients. Ongoing research has provided some insight into the pathophysiology and clinical manifestations, and new therapeutic options have recently become available for some types of pulmonary hypertension. In order to provide optimal care for an individual patient it is mandatory to establish the type and severity of the pulmonary hypertension in each patient. The diagnostic protocol used in our hospital is presented along with a description of two case histories. An algorithm of the different therapeutic strategies now available is given as well as recommendations for follow-up.