A case of metastatic epithelioid malignant peripheral nerve sheath tumor (EMPNST) in lung was admitted to Nanjing Drum Tower Hospital, Affiliated Hospital of Nanjing University Medical School. A 40-year-old women was admitted after invalid treatments in a local hospital for no obvious cause of cough and white sputum for half a month. X-ray and CT showed multiple nodules in the right middle lung. The patient had pale brown plaques throughout the body at birth and had a surgery in the right leg mass of neurofibroma, while the tumor recurred and gradually increased in size. MRI showed multiple clusters of mixed signals in the soft tissue of the right tibia and fibula. Subsequently, lump resection was performed. Microscopically, the tumor cells were arranged in a nodular and solid distribution and infiltrative growth with abundant cytoplasm of epithelioid, oval and signet ring-like cells. The tumor cell showed oval, spindle, vacuolar polymorphic nuclei and obvious eosinophilic nucleoli with visible pathological mitotic figures. Immunohistochemistry revealed the tumor cells were positive for Vimentin and focal positive for EMA and S-100. The proliferation index labeling was 30%. INI-1, LCA, TTF-1, SOX-10, CD68, HMB45 were negative. The tumor cells of lower extremity were diffusely positive for S-100 and SOX-10, focally positive for CD34, while CK-pan, EMA, Desmin, SMA, MyoD1, and LCA were all negative. Without radiotherapy or chemotherapy, the patient died due to tumor metastasis after three months later. Metastatic EMPNST was very difficult to be diagnosed and needed to be differentiated from other related epithelioid tumors by clinical history, immunohistochemical analysis and, if necessary, molecular examination.