Also called as Abrikossoff's tumor, the granular cell tumor (GCT) can occur in any part of the body, most of them occur in the head and neck especially in the tongue, cheek and palate. Almost three fourth of the lesions seen in head and neck are present over the tongue, oral mucosa or hard palate. Both benign and malignant lesions have been reported; although occurrence of malignancy is rare, comprising of 2% of all granular cell tumors. Here we present a case report of granular cell tumor of tongue at two different sites in a 17-year-old girl with a brief review of literature on granular cell tumors. Although oral GCTs exhibit a benign behavior they may demonstrate a wide variety of features and architectural patterns. Rather than a true neoplasm, GCTs could be regarded as lesions that reect a local metabolic or reactive change.