Langerhans cell histiocytosis (LCH) is a rare systemic and heterogeneous disease secondary to proliferation and diffuse infiltration of immature CD1a-positive dendritic cells, also known as Langerhans cells. LCH affects predominantly paediatric patients and is rarely diagnosed in adulthood. Despite its worldwide prevalence, most reported cases are found in the Japanese population. There is no consensus regarding treatment strategy due to the low incidence of this disease and the diversity of symptoms that appear. An integrative literature review was conducted based on the PubMed database using MeSH terms ‘Langerhans’, ‘histiocytosis’ and ‘adult’. The present report describes a case of a successfully treated LCH-induced central diabetes insipidus (uncommon presentation in adult patients) as well as an updated review of current evidence published on this matter.