Persistent coughing and bloody sputum developed in a 75-year-old man with mycosis fungoides. Chest X-ray films on admission showed combined patterns of alveolar and interstitial shadows in both lung fields. Histological examination of the transbronchial lung biopsy specimen revealed patchy infiltration of mycosis fungoides cells in alveolar septa. VEPA chemotherapy was ineffective against skin lesions and caused bacterial pneumonia, but combined treatment with etoposide (200 mg, once a week) and prednisolone (30 mg, 3 days per week) resulted in remission. The patient has been taking oral etoposide and prednisolone regularly, and has been free of skin and pulmonary lesions for two years. Combined therapy with low-dose etoposide and prednisolone may be worth trying in cases of advanced mycosis fungoides with pulmonary involvement.