Targeted therapies are effective in non-small cell lung cancer (NSCLC) patients with driver gene mutations. Chemotherapy combined with immunotherapy is also a common treatment strategy in lung cancer. However, in previous studies, patients with ALK (Anaplastic Lymphoma Kinase) rearranged had a low response to immune checkpoint inhibitor (ICI) and the role of immunotherapy in ALK-positive NSCLC patients is unclear. Here, we report a case of a young man with ALK rearranged who demonstrated a complete response to anti-PD1 combination with chemotherapy, which suggests some ALK-rearranged patients with high expression of PD-L1 may permanently benefit from immunotherapy.