Pulmonary hypertension carries a grave prognosis during gestation with maternal mortality rates as high as 30–50%, even in patients with a good pre-pregnancy functional status. A case of a successfully managed pregnant woman with severe pulmonary hypertension, due to a surgically repaired atrial septal defect, is reported. She was admitted at 29 weeks of gestation with severe dyspnea at rest, orthopnea, tachypnea, cyanosis and edema of the extremities (functional class IV). On oxygen, her arterial blood had a pH of 7.25, an oxygen partial pressure of 60 mm Hg and a carbon dioxide partial pressure of 60 mm Hg. A continuous and gradual improvement of her condition was noticed with prompt therapy including bed rest, 02 administration by face mask, digitalis, corticosteroids and diuretics. The stabilization of her condition (functional class II), allowed an uneventful cesarian section at 31 weeks of gestation, under epidural anesthesia, giving birth to a premature neonate, weighing 1,600 g. The patient died 1 year later from severe cardiopulmonary insufficiency due to the gradual progression of her severe pulmonary disease. In conclusion, prevention or interruption of pregnancy should be recommended strongly for women with pulmonary hypertension. However, if a woman, despite medical advice, chooses to continue her pregnancy, she can benefit from a prompt and well-balanced management, even in the presence of severe impairment of her functional status.