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Congenital gingival granular cell tumours are rare lesions which have only occasionally been reported in the UK. Clinical features are of a benign lesion which occurs almost exclusively in newborn, Caucasian females and the anterior maxilla is the commonest site. Treatment consists of local excision and is curative.The terminology concerning this condition has been rather confused because of uncertainty regarding the histogenesis of these tumours and the similar histological appearance to adults granular cell myoblastoma occurring at other intraoral sites. The exact histogenesis of these tumours remains unsolved and they may be hamartomata. We describe a new case occurring within the UK, which illustrates many of the common clinical features of the condition, with an accompanying literature review.