MET is a member of the receptor tyrosine kinase and proto-oncogene playing a major role in tumor development and metastasis. Nonsynonymous mutations in the MET gene have been rarely described in sarcomas (<2%). The N375K variant has been reported as a somatic variant in two cases of NSCLC. It has been also reported in a single family with familial EGFR-mutant lung adenocarcinoma. This variant lies in the non-kinase domain of the protein. Further functional studies showed this mutation reduced the binding affinity of MET for its ligand, hepatocyte growth factor (HGF), damaging subsequent cellular processes including proliferation, motility and tumorigenicity. In ClinVar it is reported as a germline variant of unknown significance (https://preview.ncbi.nlm.nih.gov/clinvar/variation/572621/). The activity of MET inhibitors in tumors with non-kinase domain MET mutations is not yet known. The clinical significance of this mutation in this tumor is uncertain. Clinical correlation is recommended.