Kyphoscoliosis can be complicated by Pulmonary Hypertension (PH) and/or right heart failure in the presence of alveolar hypoventilation. PH has been recognized as a lethal complication of kyphoscoliosis that can shorten patients’ lives across age groups. However, we have only few opportunities to encounter a patient with kyphoscoliosis in a single center in modern times and thus have little recognition about PH complicating kyphoscoliosis. Furthermore, even if we encounter a kyphoscoliotic patient with PH, no guidance or guidelines are available for reference. Recently, we have reported the successful outcome of a yearlong treatment of PH in a patient with kyphoscoliosis-related alveolar hypoventilation who had undergone Non-Invasive Positive Pressure Ventilation (NIPPV). НLs review summarizes the current concept and possible treatment option for PH in kyphoscoliosis-related alveolar hypoventilation, which we have obtained through our experience with the present case.