Kyphoscoliosis is a combined spinal deformation, which leads to a decrease in the volume of ‘working’ lung tissue with the development of alveolar hypoventilation and hypoxic vasoconstriction of the pulmonary arteries. These changes in a small percentage of cases lead to increases in pulmonary artery pressure and pulmonary vascular resistance. The pathogenesis of pulmonary hypertension in kyphoscoliosis shows resemblance to pulmonary hypertension in the setting of obstructive sleep apnea or hypoventilation in the presence of obesity. Patients with already present pulmonary hypertension may theoretically be candidates for standard pathogenetic therapy, but there is currently no evidence of the effectiveness of this treatment.