Introduction:
Malignant peripheral nerve sheath tumor (MPNST), formerly known as neurogenic sarcoma or malignant neuroblastoma, rarely occurs in the head and neck area and can mimic benign proliferative lesions. The tumor develops from the peripheral nerve cells and can occur on the surface of the skin neurofibromas in von Recklinghausen disease or de Novo.

Aim:
The aim of this paper was to present the case of the 86-year-old female patient with MPNST in the soft tissues of the right cheek.

Case study:
86-year-old female patient was admitted due to the right cheek tumor growing for about a month of the size 5 × 5 cm that caused significant asymmetry of the face.

Results and discussion:
The patient was diagnosed on the basis of the history, craniofacial visualization, histopathological examination of the tumor sample and referred for surgical treatment and possible adjuvant radiotherapy. Extensive resection of the tumor was followed by a reconstruction of buccal defect with regional lobes. The postoperative course was uneventful. Adjuvant radiotherapy was not performed due to a number of aggravating systemic illness and old age of the patient. She is the subject of constant surgical–oncological follow-up medical care. No local recurrence or distant metastases were observed during follow-up.

Conclusions:
MPNST rarely occurs in the head and neck region. This kind of sarcoma can cause problems in the process of diagnosis and therapy. Resection is the primary treatment in case of MPNST. Due to high incidence of local recurrence and distant metastases, the patients treated for MPNST require continuous outpatient follow-up after the treatment.