Background: Myoepithelioma is a rare benign salivary gland tumour, localized most frequently in the parotid and in minor intraoral salivary glands. There have been only four cases of myoepithelioma in children and adolescents reported in the English-language literature, all of them involving the plasmacytoid variant.
Case report: A 13-year-old boy, complained of a painless nodule of the palate. Incisional biopsy was performed and revealed large plasmacytoid cells with a round and eccentric nuclei. The diagnosis was consistent with myoepithelioma, plasmocytoid variant. Tumour cells were positive for cytokeratins, vimentin and S-100 protein. Surgical resection was performed and no evidences of tumour recurrence were observed after 6 years of the treatment.
Conclusion: Myoepithelioma is a very rare tumour in children and apparently presents a good prognosis, similar to occur in adult patients.