Abstract: A 27-day-old infant presented with rash, fever, hepatosplenomegaly, anemia, thrombocytopenia, and gastrointestinal bleeding in the postnatal half a month. According the features of skin biopsy and immunohistochemistry, the final diagnosis was neonatal multi-system Langerhans cell histiocytosis. The patient was successfully treated with chemotherapy of prednisone and vincristine actively. Follow up until 11 months old, good weight gain and appetite, multiple blood tests, liver and kidney function indicators are normal, and the condition is stable.

Key words: neonatal multi-system Langerhans cell histiocytosis, rash, fever, thrombocytopenia, gastrointestinal bleeding