To describe the clinical presentation of a granular cell tumour ( GCT ) in an orthodontic patient, as well as discuss its aetiology and treatment of choice. We present a case of GCT of the tongue in an otherwise healthy 17‐year‐old male patient along with a brief review of literature on GCT s. The lesion was surgically excised and orthodontic treatment was successfully finalised. Clinically, GCTs are indistinguishable from other benign connective tissue and neural tissue neoplasms and may be found in any site, with cases commonly involving the gastrointestinal system, breast and lung. However, over 50% of cases involve the head and neck, with the tongue being the most frequently involved site (65–85% of oral GCTs ). GCTs demonstrate a close anatomical relationship with peripheral nerve fibres and demonstrate the presence of myelin and axon‐like structures thus lending credence to their neural origin. The treatment of choice for GCTs is conservative surgical excision. Because GCTs present with a potential for recurrence, follow‐up is recommended. While the primary focus of orthodontic treatment is the position of the teeth within the orofacial complex, the health and wellness of the patient and his/her surrounding oral tissues always take precedence. This case demonstrates the importance of routine physical examination of the intraoral and extraoral tissues during routine orthodontic care.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106806/1/ors12055.pd