Gastrointestinal Stromal Tumors (GISTs) are mesenchymal tumors that are arises from the wall of the gastrointestinal tract and is throught to originate from the Interstitial Cells of Cajal (ICC), which regulate the motility of the gastrointestinal tract. More rarely neoplasms with histology and immunohistochemistry similar to gastrointestinal stromal tumors may ocur outside the gastrointestinal tract and are
so-called Extra-gastrointestinal Stromal Tumors (EGISTs). Surgical removal is the gold standard treatment for non-metastatic Extragastrointestinal stromal tumors. We reported a very rare case of ekstragastrointestinal stromal tumor arising from the vulva in this article.