Granular cell tumor (GCT) also known as Abrikossoff’s tumor is a benign neoplasm that is usually seen in the fourth to sixth decades of life with slight female preponderance. It is most frequently seen in the oral cavity, skin, and subcutaneous tissue. Gastrointestinal tract involvement is uncommon, in which esophagus is the most commonly affected site. There are case reports of GCT in stomach, appendix, colon and rectum. In this article, we report a case of GCT involving cecum. The cell of origin in GCT is controversial. There are various pools of thoughts regarding its histogenesis, the details of which are reviewed in this article with emphasis on the diagnostic difficulties encountered in this tumor.