Aim To report a case of non-neural granular cell tumor (NN-GCT), an uncommon neoplasm, with only six studies worldwide describing cases involving the oral cavity. Methods A 26-year-old male patient with an erythematous, firm, polypoid nodule in the floor of the mouth that exhibited areas of ulceration and mild bleeding to the touch. A biopsy was performed to aid in the diagnosis. Results Based on the histopathological and immunohistochemical results (vimentin +, CD68 +, S100 -), the diagnosis was compatible with S100-negative (primitive polypoid non-neural) granular cell tumor. No recurrence was observed over two years of follow-up. Conclusion The diagnosis of NN-GCT is extremely challenging because this tumor shares histological and immunophenotypic features with many benign and malignant tumors. Although oral NN-GCT may exhibit unusual and atypical histological features, it has an indolent behavior. Thus, until more cases of oral involvement are reported, complete resection and close follow-up are recommended. nonneural non neural NNGCT, NNGCT NN GCT , (NN-GCT) neoplasm cavity 26yearold yearold 26 year old erythematous firm touch vimentin + CD CD6 S S10 -) S100negative Snegative negative primitive followup. followup follow up. up tumors behavior Thus reported recommended (NN-GCT 2 S1 -