Objective To recognize the cases of enteropathy-type T-cell lymphoma (ETCL) with pulmonary complications primarily appeared. Methods Two cases of ETCL with pulmonary complications primarily were collected and relevant literatures were reviewed. The clinical features, diagnosis and treatment of the disease were analyzed. Results ETCL with pulmonary complications primarily appeared was so rare that it was difficult to be diagnosed. The main clinical features were repeated irregular fever and cough with abdominal symptoms which may be intestinal hemorrhage or perforation in the later period. Confirmed diagnosis depended on pathology and immunohistochemistry following laparotomy since it tended to be hard by fiberoptic bronchoscope or percutaneous lung biopsy. There was no consensual treatment currently. Conclusions The possibility of systemic disease should be prospected when pulmonary complications primarily appear with the inefficacy of conventional therapy and the negative results of the biopsy for several times. More attention could be paid to the diagnosis of ETCL especially when intestinal hemorrhage or perforation occurs. The earlier diagnosis and treatment are urgently required to improve the prognosis of these patients.