Pulmonary alveolar proteinosis is a nonspecific alveolar response to various forms of injuries. Many cases have been associated with hematologic malignancies. The most commonly reported complication of pulmonary alveolar proteinosis is superinfection with fungal, bacterial, and viral infections. We report a case of a 33-year-old man with alveolar proteinosis complicated by Mycobacterium avium-intracellulare complex infection. The additional presence of diffuse lymphocytic interstitial pneumonitis is an association that has not been previously reported.