The objective of this report is to describe a case of granular cell tumor of the anal border and to review the most relevant topics of the literature on the subject. Ours is a female patient, 57 years old, with an asymptomatic nodule in the anal border for 2 years. Surgical excision was performed, with a histopathological diagnosis of granular cell tumor. The first description of this tumor was carried out in 1926 by Abrikossoff. The techniques of immunohistochemistry and electron microscopy allowed us to determine its origin in Schwann cells. These are rare tumors, most often diagnosed between the 4th and 6th decade of life and, in general, are benign formations - only 2% of them are malignant. These tumors can occur in any part of the body, although they are more common in the oral mucosa, dermis and subcutaneous tissue. The treatment solely by surgery has a curative effect, and its recurrence is unusual. The location in the anal/perianal area occurs even more rarely, and we found only 48 cases previously described in the literature.