Introduction: Gastrointestinal stromal tumors are very rare, representing 0.1% to 1% of gastrointestinal malignancies. Gastrointestinal stromal tumors outside the digestive tract comprise a very small fraction of all gastrointestinal stromal tumors, and their most common locations are the omentum, the mesentery, and, in few cases, the rectovaginal septum. Despite their low incidence, extragastrointestinal stromal tumors are potentially malignant tumors and they present a high rate of recurrences. This, added to the fact that they are often asymptomatic until advanced stages, determines an unfavorable prognosis.
Case Report: We report a case of gastrointestinal stromal tumor located in the rectovaginal septum, which recurred after local excision; hence, a reintervention was needed.
Conclusions: A correct differential diagnosis between extragastrointestinal stromal tumors and other similar pathologies such as leiomyomas or schwannomas is imperative based on their histology and immunohistochemistry. The correct diagnosis of extragastrointestinal stromal tumors allows to start adequate treatment and follow-up, preventing recurrence that determines their poor prognosis.