Introduction: Langerhans' cell histiocytosis is a proliferative histiocytic disorder of unknown cause originating from dendritic cells.
Case presentation: The authors report a case of Langerhans' cell histiocytosis in a 48-year-old man with multisystemic disease presentation, including liver involvement.
Conclusion: Hepatic involvement is an uncommon feature in this rare disease and there is no consensus on the most effective therapeutic approach.