A 36-year-old man was admitted with cough and sputum. He had cafe-au-lait spots and multiple subcutaneous neurofibromas. Chest X-ray revealed multiple emphysematous bullae in bilateral upper lobes and a tumor in the bulla of the right upper lobe. Needle aspiration biopsy of the tumor showed small cell carcinoma. Although chemotherapy and radiation resulted in decrease in tumor size, it subsequently increased in size and he died 11 months after admission. Including this case there have been 7 reports of Recklinghausen's disease associated with multiple lung cysts and 8 reports with lung cancer in Japan. However, cases with lung cysts and cancer are very rare. The cancer of this case was considered to be associated with emphysematous bulla rather than Recklinghausen's diseases.