To improve the diagnosis and treatment of pulmonary alveolar proteinosis, clinical data for the first successfully treated case of pulmonary alveolar proteinosis with severe hypoxemia by large-capacity whole lung lavage in our hospital were analyzed, and relevant literatures were reviewed. A 35-year-old Han male initially presented two years ago with increasing cough and dyspnea was admitted to our hospital. Admission examination revealed severe hypoxemia, interstitial lung disease, and heavy protein deposition in the alveoli by lung biopsy. The patient received large-capacity whole-lung lavage in the operation room under general anesthesia and treatment of granulocyte-macrophage colony stimulating factor (GM-CSF). The patient's symptoms of dyspnea were alleviated markedly, and radiological findings improved and A-aDO2 decreased.