Here, a 50-year-old man was finally diagnosed with MPNST according to histopathology, immunohistochemistry and NGS results. This patient harbored a novel TJP1-ROS1 fusion and NF1 germline mutation identified by NGS, whom achieved stable disease (SD) under crizotinib treatment....However, after crizotinib continued treatment for 4 months, the patient's disease progressed. Soon after, the patient stopped crizotinib treatment and died in home.