Pulmonary alveolar proteinosis (PAP) is rarely seen in Taiwan. According to the literature the incidence is one in two million with a male to female ratio of 3 to 1. The pathohistology of PAP is associated with the filling of the alveoli by a proteinaceous material. PAP can be divided into primary and secondary forms. PAP was once diagnosed by open lung biopsy, but since the introduction of bronchoalveolar lavage, open lung biopsy is less common now for the diagnosis of PAP. The most definite and effective therapy for PAP is whole lung lavage (WLL). Therapeutic WLL is performed under one-lung general anesthesia with a double lumen tube (DLT). This procedure is associated with three problems: 1. hypoxia. 2. circulatory disturbance. 3. difficulty in positioning the DLT. We present a case undergoing WLL in our hospital who had contracted pulmonary TB was diagnosed as a case of PAP by open lung biopsy. She was convinced to receive WLL because of intolerance of daily activity. The result of the left lung lavage was excellent, but the attempt on the other lung at a later time was cancelled because of difficulty in positioning the DLT due to tracheal stenosis. Here we would like to give an account of our management and discuss about PAP and WLL after literature review.