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Langerhans cell histiocytosis (LCH) identifies a spectrum of disorders that are classified according to the organs involved (single- or multi-organ disease) and the presence or absence of organ failure. Patients younger than 2 years with a multisystem disease have a significantly higher mortality rate than older children.1 Nail involvement in LCH is extremely uncommon and rarely reported. To our knowledge, 18 cases have been documented in the literature to date. According to most cases reported, it is thought to indicate a poor prognosis.2, 3, 4, 5, 6 We report the case of a child with nail lesions, which led to a diagnosis of LCH.