Lung transplantation has been successfully used in the treatment of patients with end-stage pulmonary disease and adequate cardiac function. We report about a 32-year-old man with pulmonary alveolar microlithiasis who underwent sequential bilateral lung transplantation. Preoperative hemodynamic studies revealed severe pulmonary hypertension; the right ventricular ejection fraction was 0.27. Eighteen months postoperatively, he continues to do well with normalized pulmonary and cardiac function and without clinical or histopathologic signs of graft rejection.