Salivary gland neoplasms (SGNs) in children are uncommon. Epithelial SGNs (ESGNs) comprise the majority (95%), with the remaining being mesenchymal SGNs (MeSGNs). Pleomorphic adenoma is the most frequently encountered benign neoplasm, mucoepidermoid carcinoma is the most frequent malignant ESGN, and rhabdomyosarcoma is the most frequent malignant MeSGN. ESGN presents in the second decade, whereas MeSGN presents in the first and second decades. Swelling without pain or neurological signs is the main presentation of both benign and malignant neoplasms. Making an accurate preoperative histological diagnosis is important, so a needle biopsy or a perioperative frozen section is useful when there is doubt about the disease status of the patient; the excised tumour margin is also important. Surgical excision should aim to achieve clear margin excision in benign and malignant ESGNs, minimising the need for adjuvant radiotherapy and maximising the long-term likelihood of patient cure. Benign ESGNs are uncommon, and excision is curative, whereas malignant ESGN and MeSGN should be managed by a multidisciplinary paediatric oncology team.
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