Abstract Langerhans cell histiocytosis (LCH) has diverse presentations which can bring it before all physicians regardless of specialty. A retrospective audit was undertaken at Westmead Hospital, Sydney, Australia, to ascertain the incidence, epidemiology and clinical features of patients with LCH over a 10‐year period (1994–2004). A total of 12 patients was identified (six male, six female). Eleven patients had involvement of the skeletal system, three of the patients had pulmonary LCH and only one patient presented with soft tissue involvement (nose and antrum). Three patients had diabetes insipidus. Our results are consistent with that noted in the published literature and confirm the low incidence, diverse nature of presentation and the differing treatment strategies available for this rare and yet interesting condition. (Intern Med J 2005; 35: 622–625)