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Extra-gastrointestinal stromal tumor(EGIST) is a relatively rare mesenchymal tumor occurring outside the gastrointestinal tract. The immunophenotype, histological morphology, gene mutation sites and mutation frequency of EGIST are similar to GIST. EGIST lacks typical clinical features and is not easily diagnosed. Due to the small number of reported cases, there is no unified risk assessment standard. A case of EGIST originating in vaginal wall is reported in this paper, which diagnosis depended on clinical data and immunohistochemical markers. Literature reviewing suggestes that the incidence of EGIST is low and patients with EGIST have worse prognosis.