Pulmonary enteric adenocarcinoma is a primary pulmonary adenocarcinoma with similar histology to colorectal adenocarcinoma.Its pathogenesis is not clear and may be related to the intestinal type differentiation of cancer cells.The main clinical symptoms of pulmonary enteric adenocarcinoma are similar to those of ordinary pulmonary adenocarcinoma, including cough, expectoration, sputum mixed with blood and so on.The imaging findings are also similar.The missed diagnosis and misdiagnosis rate of pulmonary enteric adenocarcinoma is high.The serum levels of CEA and CA199 in Pulmonary enteric adenocarcinoma were significantly higher than those in ordinary pulmonary adenocarcinoma, but there is no statistical difference compared with lung metastatic colorectal cancer.The intestinal type differentiation component of pulmonary enteric adenocarcinoma is more than 50%, and some of them express intestinal type differentiation markers.Pathological examination is an important means for the diagnosis of pulmonary enteric adenocarcinoma.Next-generation sequencing is helpful for diagnosis.The treatment principle of pulmonary enteric adenocarcinoma is the same as that of ordinary pulmonary adenocarcinoma.The chemotherapy regimen for ordinary pulmonary adenocarcinoma is effective for pulmonary enteric adenocarcinoma.The most common genetic mutation in pulmonary enteric adenocarcinoma is the KRAS mutation, which has certain hints for its targeted therapy.