Pulmonary alveolar lipoproteinosis, described for the first time in 1958 by Rosen SH, Castleman B and Liebow AA, is a rare pathological condition characterized by alveolar accumulation of lipoproteinaceous material. It is the result of macrophages impairment to rid the alveolar spaces of spent surfactant. This condition involves a restrictive function of pulmonary tissue, reflected in gas exchange impairment and respiratory symptoms of variable severity. Until now, about 410 cases have been reported in the literature. From these cases, 90% were represented by primary type of pulmonary alveolar lipoproteinosis. We present the case of 37-year-old male patient admitted in the Department of Internal Medicine, Emergency County Hospital, Constanta, Romania, with progressive exertional dyspnea, dry cough and perioral cyanosis. The clinical symptoms started three months before hospital admission. Based on clinical findings and imaging features, the primary pulmonary alveolar proteinosis diagnosis has been suspected. Uncharacteristic serous aspect of fluid resulting from bronchoalveolar lavage required open lung biopsy. Pathologic examination of pulmonary slice revealed features consistent with the diagnosis of pulmonary alveolar lipoproteinosis associated with emphysematous foci. The peculiarity of this case lies in the association of two pathological conditions, each of them requiring different pathways.