Granular cell tumor (GCT) is a common neoplasm that can occur in any part of the body, including the orofacial region with controversial origin. The tumor is usually benign, but there are case reports in which the tumor shows a locally aggressive behavior, malignancy and distant metastases. The most widely accepted hypothesis is that granular cell tumor arises from the altered metabolism of Schwann cells. The tumor is typically asymptomatic and appears as a nodule that does not exceed 3cm. This article brings forth a case of GCT occurring in a 42 years old male patient on the lateral border of the tongue.