Nail involvement in Langerhans cell histiocytosis is uncommon and is said to indicate a poor prognosis. We describe a 2-year-old boy with onycholysis, subungual hyperkeratosis, and hemorrhages on his fingernails. He also had hepatosplenomegaly and pulmonary involvement. The diagnosis of Langerhans cell histiocytosis was made by histopathologic examination of skin and liver.The role of nail involvement as an unfavorable prognostic sign is still unclear and this paper concludes that nail involvement in Langerhans cell histiocytosis is a possible sign of multisystemic involvement.