NF2 and neoplasm: Our case showed the sustained clinical benefit of anti-PD-1 monoclonal antibody in the uncommon tumor subtype harboring an <i>NF2</i> mutation as a first-line therapy after non-radical surgery despite the heavy tumor burden.<h4>Conclusion</h4>Our case indicated that ICIs are warranted as first-line monotherapy in MPNSTs given the possibility of life quality improvement and durable clinical benefit.