Granular cell tumor is a rare tumor that can occur at any location. The most frequent one is the buccal mucosa especially on the tongue, dermis and subcutaneous tissue. Few cases were reported to occur in the perianal area. Although it is an almost always-benign entity, it can be associated to an alteration of the patient’s quality of life. Although a malignant variant of this tumor exists, it barely presents 2% of all cases. The basic treatment is surgical excision. In our work, we report a case of perianal granular cell tumor in a 52 years old female evolving during a period of one year. In our work, we also discuss the most important epidemiological, clinical, pathological, therapeutic and prognostic features of this rare entity.