Abstract Langerhans' cell histiocytosis (LCH)--once called histiocytosis X--is a complex reticuloendothelial disease that often involves the head and neck. We discuss the current nomenclature of this disease and review its pathologic and clinical characteristics, with particular emphasis on the role of the head and neck surgeon. LCH can be challenging to diagnose, and the otolaryngologist must be familiar with its varied presentations. Because LCH usually responds well to medical therapy and extensive resection can easily cause more morbidity than the disease itself a minimalist approach to treatment usually provides the best outcome. We also discuss the case of a 9-month-old girl with LCH who presented with aggressive head and neck disease.
