MTT was confirmed by postoperative pathology and immunohistochemistry.<h4>Conclusion</h4>Despite the preoperative diagnosis of hepatocellular carcinoma with a rising serum AFP level, typical CT and MRI findings, histopathology assessment showing MPNST with rhabdomyosarcoma differentiation confirms the diagnosis of primary hepatic MTT. The gene discussed is AFP; the disease is malignant peripheral nerve sheath tumor.