Lung cancer is a kind of malignant tumor with high morbidity and mortality in the world. The most common subtype is non-small cell lung cancer (NSCLC) which accounts for 85% in lung cancer. Human epidermal growth factor receptor-2 (HER2) is one of the most important members of receptor tyrosine kinase (RTK). In NSCLC, HER2 gene is mainly expressed in three phenotypes: HER2 mutation, HER2 amplification and HER2 overexpression. At present, the drugs targeting HER2 mutation, including tyrosine kinase inhibitors, monoclonal antibodies and antibody-drugs conjugates, have shown clinical efficacy in HER2 mutation NSCLC patients. However, the clinical efficacy of immunotherapy is limited. In this review, we discussed the progress of treatment in HER2 mutation NSCLC, so as to provide a theoretical basis for further clinical treatment strategy of advanced non-small cell lung cancer patients with HER2 mutation.