A 51-year-old woman presented with complaints of dyspnea, fatigue, and non-productive cough. Chest X-ray showed bilateral lung infiltrates. Nonspecific air-space consolidation on anterior segment of the right lower lobe, bilateral bronchiectasis and infiltrates, patchy ground-glass opacities, and interstitial thickening were reported on thorax computed tomography which was non-responsive to antibiotics. After tru-cut biopsy which only revealed a single granuloma in a particular area, alveolar septal thickening and fibrosis, slight chronic inflammation with findings of congestion, lung involvement was considered to be associated with nonspecific interstitial pneumonia. The nails on all fingers displayed yellow discoloration with mild edema in the face and the legs. The final diagnosis was yellow nail syndrome. Short-term clarithromycin and long-term oral methylprednisolone with vitamin E treatment were successful. After 4 months, all components of the syndrome almost completely regressed