BACKGROUND The pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of lipoprotein material in the alveoli. It is classified as primary, secondary and congenital. The primary form, of autoimmune origin, is characterized by antibodies against granulocyte-macrophage colony stimulating factor (GM-CSF). The aim of this article is to present a case of PAP with adequate response to treatment with bronchoalveolar lavage (BAL) and GM-CSF. METHODS A 28-year-old female patient who started with mild to moderate effort dyspnea, distal cyanosis and lower respiratory tract infection. We found restrictive pattern respiratory function tests, chest X-ray with bilateral nodular reticulum pattern and high-resolution tomography with ground glass image and bronchiectasis, besides video bronchoscopy with inflammatory process. The open biopsy revealed data of alveolar proteinaceous material and mononuclear infiltrate. Treatment was given with BAL and GM-CSF which was suspended by dermal reaction. The patient had a satisfactory evolution and is currently asymptomatic. CONCLUSIONS The present case had clinical, imaging and histological manifestations for the diagnosis of autoimmune PAP with a satisfactory response to treatment. Although PAP is a low prevalence entity, the diagnosis and therapeutic options must be taken into account, including BAL and GM-CSF, since this factor is required for surfactant factor homeostasis.