A case of pulmonary alveolar proteinosis associated with tuberculosis of the lung is reported. The patient had fever, cough, and pulmonary cavities. Sputum cultures for Mycobacterium tuberculosis were positive on three occasions. Thirty-three months later, diffuse bilateral lower lobe infiltrates developed. An open lung biopsy revealed filling of the alveoli by a periodic-acid-Schiff-positive amorphous granular material. There is a significant association between this disease and various infectious and fungal agents, but its association with tuberculosis is rare. It has been reported only three times before in the English literature. This appears to be the fourth documented case with this association. A review of the literature with special emphasis on tuberculosis associated with alveolar lipoproteinosis is presented. The electron-microscopic findings are also described.