Background <p>Malignant peripheral nerve sheath tumors (MPNSTs) rarely occur on the top of the head. MPNSTs are usually characterized by invasiveness, recurrence, and metastasis.</p> Case presentation <p>Herein we report a 44-year-old female patient who received multidisciplinary collaborative treatment for a giant MPNST on the top of her head. The patient was initially evaluated in the Departments of Neurosurgery and Burn Plastic Surgery at our hospital. She then underwent elective surgery after psychological counseling. Surgeons from the Departments of Neurosurgery and Burn Plastic Surgery collaborated during the surgery. Specifically, surgeons from the Department of Neurosurgery excised the lesion with meticulous hemostasis under general anesthesia. Then, surgeons from the Department of Burn Plastic Surgery performed personalized skin grafting following a predetermined regimen, which consisted of the following steps: a razor-thin graft was harvested from the anterior thigh and transplanted to the target site to repair the scalp defect; and vacuum sealing drainage was performed. The patient was followed for 6&#xa0;months postoperatively and had no signs of a recurrence. The patient was very satisfied with the treatment and outcome.</p> Conclusions <p>Complete surgical resection is recommended for a giant MPNST on the top of the head. Patients should undergo a comprehensive evaluation of disease severity, general health status, and mental state preoperatively. The treatment regimen can be personalized through a multidisciplinary approach. Intraoperative blood loss should be closely monitored, along with regular postoperative follow-up evaluations to confirm treatment efficacy.</p>