Peripheral primitive neuroectodermal tumours, which belong to the Ewing sarcoma tumour family, are extremely rare. These tumours are highly aggressive and are known to have a poor prognosis. Immunostaining with at least two neural markers and evidence of an abnormal t(11;22)(q24;q12) translocation are hallmark features in this diagnosis. We present the first reported case of peripheral primitive neuroectodermal tumour to occur in the tongue.