A case of epithelioid sarcomain of intracranial and extracranial communication involving the occipital lobe and skin is reported. A 22-year-old male has been presented with headache and unclear vision for more than one month. He had a history of surgery for right occipital subcutaneous epithelioid sarcoma.Pathology showed that the tumor was multinodular with fibrous tissue between the nodules. A pseudomembrane can be formed when the fibrous tissue was squeezed. Hemorrhage, granuloma like structure and blood metastasisand can be seen in the tumor tissue. Immunohistochemical results showed the tumor cells were positively stained for CK-pan, CD34 and EMA,but negative for ini-1. Medical history, clinical histopathology and immunohistochemistry supported the recurrence of epithelioid sarcoma with hematogenous brain metastasisdiagnosis.