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Case: A 55-year-old man attended our hospital because of a mass at the above his right eye. Cranial MRI showed an enlarged mass 5cm in diameter with intracranial invasion and metastatic brain tumors. The mass was pathologically diagnosed as adult T-cell leukemia/lymphoma (ATLL) because of a high HTLV-1 antibody titer, and radiation therapy was started. Furthermore, since we noted multiple cervical lymphadenopathy, we performed an additional biopsy, which showed poorly-differentiated adenocarcinoma. The primary lesion was the lungs, with bilateral pleural effusion and lymphangitis carcinomatosis. We diagnosed stage IV primary lung cancer and started chemotherapy. However, he developed dyspnea due to pleural effusion and his performance status gradually decreased to 3. Fifteen days after readmission to hospital, he manifested sudden respiratory failure and shock, and died the next day. Autopsy showed ATLL and extensive lung cancer with multiple metastases. There was invasion of ATLL in systemic lymph nodes, which coincided with invasion of adenocarcinoma., Conclusion: We encountered a rare case of ATLL and primary lung cancer. Accumulation of cases and further investigations are awaited.