We describe a lung disease due to AR complete CCR2 deficiency presenting in childhood with the development of PAP, marked peribronchovascular and parenchymal lymphocytosis, with peribronchiolar pulmonary fibrosis, progressive diffuse parenchymal lung cyst formation and enlargement, progressive obstructive airflow limitation, and recurrent secondary infections. This evidence concerns the gene CCR2 and pulmonary fibrosis.