MPNST is a form of cancer of the connective tissue surrounding nerves. The case of MPNST in forms of lymph node (LN) metastasis was not reported yet. Here, we report a case of MPNST in LN presenting as a neck mass. A11-year-old man visited out-patient clinic due to right neck mass. Contrast enhanced CT scan of neck revealed a 3 cm sized soft tissue mass in the right side neck level 2 superficial area. Histopathology from sono-guided needle biopsy revealed a benign spindle cell neoplasm. He underwent surgical excision of neck mass. Under general anesthesia, we identified subcutaneous mass distinguishable from parotid gland. Pathologically, a cellular tumor composed of spindle nerve sheath inLN. Immunohistochemical staining showed positive finding for vimentin, S-100, neuron specific enolase, negative for neurofilament, TLE-1, and HMB45. And final pathology was confirmed as neck metastasis of MPNST. To find the primary lesion, whole body PET/CT and MR were performed, but primary lesion was not detected. On PET and MR, metastatic LN was detected at right level I area. Secondary surgical excision was done and followed by chemotherapy and radiotherapy.