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Newborn with a rare congenital granular cell lesion of the tongue. 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.