An uncommon and aggressive type of soft tissue sarcoma that develops from peripheral nerves is known as a malignant peripheral nerve sheath tumor (MPNST). It is typically associated with neurofibromatosis type 1. Hence, this case report presents a case of a 42-year-old woman with a mass over the palmar aspect of her right hand that had been slowly growing over the previous year. Complete resection of the tumor with groin flap was performed followed by adjuvant radiotherapy. Over the last year, the patient has been monitored on an outpatient basis without displaying any signs of a local recurrence.