Objective: Primary lung enteric adenocarcinoma is a rare type of invasive lung carcinoma. This article mainly discusses the clinicopathological characteristics, diagnosis and treatment of primary lung enteric adenocarcinoma. Methods: Retrospectively analysed clinical records of 6 cases admitted in hospital from Feb 2013 to May 2016, and reviewed the literature of primary lung enteric adenocarcinoma. Results: Two patients were male and four patients were female with the age ranged from 25 to 78 years. Their symptoms consisted mainly of cough chest stuffy with 4 patients, neck mass with 1 case, dizziness nausea vomiting with 1 case; imagining scan showed mass of lung and or mediastinal, pathology form and the immunochemistry showed all positive for intestinal immune phenotypes and some positive for lung cancer immunophenotypic. But no tumor was found by gastrointestinal endoscopes; 1 case recurrence and metastasis after radical operation; 1 patient underdone palliative surgery, 1 patient with brain solitary metastasis onset received CyberKnife and without system treatment, and 5 patients underwent chemotherapy. At the end of follow-up, 4 patients died, over survival time as long as 32 months. Conclusion: The primary lung enteric adenocarcinoma was easily confused with pulmonary metastases from colorectal cancer, confirmed diagnosis need to rule out intestinal lesion, the main early treatment is surgery, and systematic treatment programs need to be further studied.