We report here a case of fatal respiratory failure developed during chemotherapy for diffuse large B cell lymphoma that occurred late after lung transplantation. 25-year- old man underwent lung transplantation from brain death donor for respiratory failure due to interstitial pneumonia at the age of 16 years old. Two years after transplantation, his respiratory function decreased gradually. Chronic lung allograft dysfunction including bronchiolitis obliterans( BOS) and restrictive allograft syndrome was suspected and immunosuppression was enhanced. Nine years after transplantation, he had abdominal pain and physical examination suggested intestinal obstruction. Small intestine endoscopy revealed an ulcer at jejunum and diffuse large B cell lymphoma( DLBCL) was finally diagnosed by biopsy. Chemotherapy was planned for lymphoma, but respiratory failure progressed just before chemotherapy. Chest computed tomography showed infiltrative shadow in right lung, so we suspected presence of lymphoma and chemotherapy was carried out. After chemotherapy, abnormal shadow in the right lung disappeared. Although chemotherapy was effective, respiratory failure progressed and he died. Pathological examination from autopsy showed mixture of BOS, diffuse alveolar damage, invasion of aspergillus and acute fibrinoid organizing pneumonia but no residual DLBCL was found in the lung.