Objectives: Langerhans' cell histiocytosis (LCH) is a rare disorder of unknown aetiology, characterised by the proliferation of histiocytic cells in various tissues and organs. The role of the otolaryngologist is important in the early and accurate evaluation, staging and diagnosis of LCH, as it may mimic more common otological disorders.
Method: We report the case of a 2-year-old child presenting with LCH and review the literature concerning this rare disease.
Results: Presentation in our case was with a common aural polyp, refractory to medical treatment, which when biopsied, revealed LCH. Radiological evaluation showed multifocal disease.
Conclusion: A sound knowledge of the disease process in vital to the otolaryngologist, thus enabling rapid diagnosis and early treatment for a commonly fatal condition.