AbstractAimWe report a rare case of a calcifying epithelial odontogenic tumour with atypical clinical presentation in an adolescent and present a literature review of this tumour in paediatric patients.Materials and MethodsA 12‐year‐old female patient presented with a painless swelling of the left mandible, with a history of 6 months. Clinical examination revealed a sessile mass with a reddish‐pink colour and firm consistency. Tomographic images showed a hypodense, unilocular lesion involving unerupted tooth #34, with a hyperdense focus in contact with the tooth crown disrupting the buccal cortex.ResultsEnucleation, curettage and peripheral osteotomy of the lesion were performed, and the impacted tooth was removed, followed by the application of autologous leucocyte and platelet‐rich fibrin matrices to the region. The patient evolved favourably with good clinical healing and evidence of new bone formation.ConclusionsDespite its aggressive potential, the conservative approach for CEOT in paediatric patients proved to be effective. Our review found reports of recurrence up to 13 years after surgery. Therefore, long‐term clinical and radiographical follow‐up is indicated.