Hypoxic pulmonary hypertension (HPH) develops in many patients with advanced chronic obstructive pulmonary disease (COPD) and aggravates their quality of life and prognosis. The most proper but as yet not satisfactory management of HPH involves COPD prophylaxis. In this article pathophysiological background to use systemic vasodilators in treatment of HPH are described, and hemodynamical and clinical results of short and long term trials are discussed. Authors explain lack of clinical benefits with currently used systematic vasodilators in HPH and indicate promising preliminary results with 5-phosphodiesterase inhibitors. The substantial role of chronic oxygen therapy and pulmonary rehabilitation in management of HPH is emphasized.