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Sickle cell disease (SCD) is characterized by repeated episodes of hemolysis and vaso-occlusion. Hemolysis is a risk factor for the development of pulmonary hypertension (PH), and currently SCD-related PH is classified as World Health Organization group 5 PH. Patients with SCD-related PH have unique hemodynamics, comorbidities, and phenotypes that may contribute to the development of PH. SCD-related PH is defined as a mean pulmonary artery pressure >20 mm Hg, a pulmonary artery occlusion pressure ≤15 mm Hg and relatively low pulmonary vascular resistance (PVR) (>160 dynes·sec/cm5 rather than the typical definition of ≥240 dynes·sec/cm5), an important distinction because of a baseline high-cardiac output state in the setting of chronic anemia and low vascular resistance. Diastolic dysfunction is common, and right heart catheterization is warranted to determine if combined precapillary and postcapillary PH is present. Thromboembolism is common among patients with SCD, and screening for chronic thromboembolic pulmonary hypertension is essential. There are few studies evaluating advanced therapies in this population. The mainstay of treatment includes targeting correction of their primary hemoglobinopathy as well as aggressive management of underlying comorbid conditions. SCD-related PH is common among patients with SCD and is associated with increased mortality. A high index of suspicion is warranted during evaluation to identify all potential factors that may be contributing to disease. [ABSTRACT FROM AUTHOR]