Pulmonary disease is the most common cause of death in patients with systemic sclerosis. This article discusses the variety of ways the lung is affected in systemic sclerosis. These include pulmonary hypertension, interstitial lung disease and other restrictive disorders, obstructive disease, pleural disease, lung cancer, and aspiration. For each manifestation we outline the current knowledge of etiology, risk factors, diagnosis, treatment, and prognosis. We also discuss the current state of lung transplantation in this population.