Objective: Gastrointestinal stromal tumors (GIST) are mesenchymal tumors derived from interstitial cells of Cajal. They are found more frequently in the stomach, small intestine while colon and rectum represent unusual sites. GIST's of the anal canal are extremely rare. Method: 43-years-old woman who presented bleeding, pain and constipation for several months. The rectal examination revealed a well defined mass located within 10 cm of the anal verge. The magnetic resonance imaging confirmed a well-circumscribed mural mass without adenopathy. The local excision of the tumor was performed for pathological study. Results: Gross examination showed a 4.5×3.0 cm fibrous mass. Histological examination revealed a spindle cell tumor with moderate atypia and mitotic count of 6 mitosis/ 50HPF. Neoplastic cells showed marked positivity for c-Kit and CD34 and negativity for muscular markers. A diagnosis of GIST with intermediate risk of aggressive behavior was made. Conclusion: GISTs of the anal canal are extremely rare with only few cases reported in the literature. We described an additional case of GIST of the anal canal with histological and immunohistochemical study.