Introduction: Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive soft tissue sarcomas in which complete surgical resection is the mainstay of therapy.
Clinical Presentation: We report a case of MPNST, a 24 year old patient with right neck painful mass of 13 cm of greater dimension, skin spots, lentigos, cutaneous neurofibromas. The neck CT scan revealed a mass with liquid and soft tissues densities.