Background: Langerhans Cell Histiocytosis is a rare clonal disorder involving CD1a+/CD207+ dendritic cells. While the head and neck region is frequently affected in paediatric LCH, otolaryngologic manifestations are not well-synthesised in the literature. These presentations often resemble common ear, nose, and throat conditions, potentially delaying accurate diagnosis and treatment.Methods: A systematic review was conducted using PubMed, Ovid Medline, Ovid EMBASE, and Cochrane Library, identifying 73 studies encompassing 713 paediatric LCH patients with otolaryngology/head and neck involvement. Concurrently, a retrospective case series identified 25 contemporary cases from a Victorian quaternary paediatric centre.Results: Among the 713 patients from the systematic review, the mean age at diagnosis was 6.6 years, with 62.7% male. Single-system unifocal disease was the most prevalent presentation. Common presentations included scalp/skull swelling, pain, cervical lymphadenopathy, otorrhoea, and external auditory canal masses. The calvarium and temporal bone were the most frequently affected subsites. Diagnosis was histologically confirmed, with CT and MRI being primary imaging modalities. Overall recurrence rate was 15.3%, higher in multisystem disease. In the statewide case series, the mean age was 8.4 years, and 72% presented with single-system unifocal disease. All cases underwent biopsy with immunohistochemical confirmation. Thirteen patients required systemic therapy, with remission achieved in all unifocal cases.Conclusion: Head and neck manifestations of paediatric LCH are common and often mimic benign otolaryngological conditions, highlighting the need for increased clinical suspicion and early biopsy. Unifocal disease typically follows an indolent course with excellent outcomes, whereas multisystem involvement is associated with higher recurrence rates. Contemporary management increasingly integrates molecular diagnostics and targeted therapies, underscoring multidisciplinary care and structured follow-up.