Granular cell tumors (GCTs) have been described as neoplasms of Schwann cell origin. They are often benign and notably uncommon in the gastrointestinal tract. Recently, their incidence has become more common, likely as a result of increased colonoscopy screenings. Very few data exist regarding their potential for malignancy, but malignant GCTs have been reported. Here, we report the case of a young female patient who was diagnosed with an atypical GCT on the ileocecal valve with an overlying tubular adenoma which was found incidentally on colonoscopy. This represents the first known report of a GCT with atypical features on the ileocecal valve, as well as a rare case of overlying adenomatous changes. Due to the paucity of cases, there is no clear modus operandi for their management. Furthermore, it is not clear whether surgical or endoscopic interventions or simple observation may be most appropriate. Further studies are needed to evaluate the potential for malignancy of this tumor, as well as its management.