Pulmonary hypertension (PH) is a severe complication of sickle cell disease (SCD). The main purpose of this study was to evaluate the prevalence of PH in an adult SCD population in Guadeloupe. Echocardiography findings of 427 patients with SCD were analyzed. Subjects with a pulmonary artery systolic pressure (PASP) >30 mm Hg, at rest, were studied. PH was found in 49 (11.5%) SCD patients. Mean age was 37 years and mean PASP was 40 mm Hg. The most frequent symptom was exertional dyspnea. Thirteen subjects (26%) had left heart diseases. In the overall study population, 22 (5%) patients died. Seven (14%) patients had PH and 15 (4%) were PH free, p = 0.006. An early PH screening is recommended in SCD patients at steady state owing to the absence of specific symptoms and to the significant mortality rate related to this complication.