Abstract: In patients with epidermal growth factor receptor (EGFR) mutation, secondary resistance to EGFR tyrosine kinase inhibitors (EGFR-TKI) is inevitable. Immune checkpoint inhibitors (ICIs) are currently the mainstay of cancer therapy, including monoclonal antibodies against programmed cell death 1 (PD-1), programmed cell death ligand-1 (PD-L1) and cytotoxic T lymphocyte-associated protein 4 (CTLA-4), which can significantly improve survival and quality of life in patients with non-small cell lung cancer (NSCLC). However, NSCLC patients with EGFR mutations have a poor response to ICIs. Studies have shown that EGFR signaling pathway may influence anti-tumor immune response and tumor immune microenvironment. This paper reviewed the effects of EGFR signaling pathway on tumor immune response in tumor immune microenvironment, related prognostic indicators, current status of immunotherapy for patients with EGFR mutation and current clinical studies.