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Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology. It can occur at any age but is more frequent in the pediatric population at young age. Because of its infiltration nature, the disease varies widely in clinical manifestation from localized to a multisystem involvement. Owing to the relative rarity of the condition and varies in clinical presentation, it remains a disease in which the diagnosis is often delayed or missed. Jaundice in LCH is a manifestation of liver disfunction. However, a case of cholestasis has been very rare described. We report an 18-months-old boy with LCH involving multiple systems whose present earlier as cholestasis and recurent fever. The patient was diagnosed following a clinical manifestation of LCH and skin biopsy that revealed Langerhans cells. The patient was in partial remission following chemotherapy and died at fourth months of chemotherapy. In conclusion, any patient presenting to pediatric clinic with jaundice must be rule out the possibility of liver involvement in LCH. Jaundice might be an earlier clinical manifestation of liver disease in LCH and can be a clue for diagnosis. Early diagnosis and proper management would contribute to a better outcome.

Cholestasis as manifestation of langerhans cell histiocytosis in an 18-months-old boy