Introduction. Pulmonary alveolar proteinosis (PAP) is a relatively rare lung disorder, probably under diagnosed, characterized by the accumulation of lipoproteinaceosus material in the lung alveoli. The primary (acquired or idiopathic) form occurs in more than 90% of all cases. Whole lung lavage is considered the golden standard of treatment. In this report, we describe a rare case of pulmonary alveolar proteinosis with severe and incapacitating respiratory, in which whole lung lavage followed the thoracotomy for spontaneous pneumothorax.