Pulmonary hypertension (PH) is a relatively frequent and severe complication of sickle cell disease (SCD). PH associated with SCD is classified as Group 5 PH. The exact pathogenesis of PH in SCD in not known. There are also very limited treatment options available at this time for such patients with Group 5 PH. Patients with SCD are predisposed to a hypercoagulable state and thus can also suffer from chronic thromboembolism. These patients can have associated chronic thromboembolic pulmonary hypertension (CTEPH), thus being classified as Group 4 PH. We present such a case of a patient with SCD diagnosed with severe PH who was found to have CTEPH and successfully underwent a thromboendarterectomy with resolution of his symptoms such as reduction of his oxygen requirements and healing of chronic leg ulcer. This case illustrates the importance of screening patients with SCD and elevated pulmonary artery pressures for CTEPH as this would offer possible treatment options such as pulmonary thromboendarterectomy and/or riociguat in this subset of patients.