Granular cell tumors (GCTs) are relatively rare submucosal tumors with an incidence of 10% in the gastrointestinal tract. In the gastrointestinal tract, the esophagus is the most common site for GCTs, while the colorectum is an uncommon site. GCTs are often found incidentally as small, submucosal tumors on esophagogastroduodenoscopy. On endoscopic ultrasonography (EUS), GCTs usually have a homogeneous hypoechoic pattern. EUS cannot adequately distinguish GCTs from carcinoid tumors. We report a case of a cecal GCT that was misdiagnosed as a carcinoid tumor by EUS and was treated by endoscopic mucosal resection, along with a review of the literature.