Congenital granular cell lesion (CGCL) is an uncommon, benign soft tissue lesion that usually arises from the alveolar mucosa of neonates. The present report describes a case of CGCL originating from the tongue of a newborn female infant. The lesion was negative for S-100 differentiating it from congenital granular cell tumour. The lesion was excised under local anaesthesia with no recurrence at 4 months.