Abstract: Lung cancer is a malignant tumor with the highest morbidity and mortality in China, and non-small cell lung cancer accounts for about 85%. Epidermal growth factor receptor mutation is one of the most common driver genes in NSCLC. In NSCLC, rare EGFR mutations are EGFR mutations other than exon 19 deletion mutation and exon 21 L858R mutation, accounting for about 7-23% of EGFR mutations. More and more rare mutations and unknown mutations are validated in recent years as the wide application of next-generation sequencing. Many rare EGFR mutations couldn’t be effectively treated due to the lack of centralized clinical data and experimental recruitment. We address the EGFR gene structure and mutation characteristics, the approaches for rare EGFR mutations detection, and summarize the treatment plans and research progress of these mutations in non-small cell lung cancer in recent years, in order to benefit clinical detection and treatment.