Epitheliod sarcoma of vulva is a very rare kind of tumor which its diagnosis cannot be readily reached; however, it is characterized by a variety of aggressive biological behaviors and poor prognosis. We report a case of vulvar epitheloid sarcoma in a 24?years-old woman who presented with protruding, mushrooms like mass on multiple area including lower abdomen, whole vulva, anus and both inguinal lesions along with distant metastasis to lung, pleura, bone and scalp. Result from histological evaluation showed malignant tumor, consistent with proximal type of epithelioid sarcoma. The main lesion was an open wound of large necrotic tissue formation with exposed bone on whole vulva. Her treatment started with palliative radiation therapy followed by adjuvant chemotherapy. Follow up imaging studies revealed a good response with partial resolution of vulvar lesion and complete resolution of pelvic bone metastasis. However, aggravation of multiple lung and pleural metastases was detected. Consequently the patient received target therapy of Pazopanib which is an inhibitor of vascular endothelial growth factor receptor (VEGFR) pathway. After two months of daily regimen of Pazopanib (400 mg) follow up imaging studies showed partial resolution of both lung and pleural metastases. Pazopanib plays a crucial role in distant metastasis of solid malignancies and brings a delay in progression of the disease.