Aneurysmal bone cysts (ABCs) are benign, vascular and cystic bony tumors which can be rapidly expanding and locally cause destruction of the surrounding tissue. ABCs in the head and neck area are usually found in patients with younger age group. ABC in the head and neck region of children may vary in presentations and severity.  ABCs are commonly found in mandible, sinonasal tract and cranium in head and neck region. Imaging and histopathological examinations confirm the diagnosis. Complete surgical excision is the treatment of choice. ABC which is secondary to underlying bone pathology like osteoblastoma may refractory to treatment. ABC in the head and neck region are uncommon and should be considered as differential diagnosis of bony and vascular lesions in the locations like mandible, sinonasal area, cranium, mastoid bone and spine. The review article includes case series, case reports and original research on ABCs manifesting in the head and neck area published in the English language. All literatures identified via Scopus, Google scholar, Medline and PubMed were analyzed individually. Articles of ABCs included according to specified eligibility criteria. The total number of articles were 78 (41 case reports; 37 cases series; 8 original articles). ABCs rapidly grow with expansile manner and result in destruction of bone and surrounding tissue in the head and neck region, so clinicians must think about this clinical entity to diagnose correctly and treat appropriately. The aim of the article is providing a comprehensive review of the ABC in the head and neck region of the pediatric patients.

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