Objective To understand the clinical features and laboratory examination and diagnosis of Langerhas cell histio-cytosis (LCH) in newborns. Methods Retrospective analysis of the clinical data of a neonatal case of LCH was performed. Results A one-day-old girl was admitted with chief complaint of eating less, less crying and skin rash for one day. The unequal sized rash was on the head, limbs, trunk, toe and oral mucosa. The skin rashes were protruding from the skin and were partially broken and crusted. Biopsy of the skin tissue showed the expression of S-100 and CD1α, consistent with the diagnosis of LCH. Conclusions When new-borns have special rash after birth, LCH should be considered. To avoid misdiagnosis, skin biopsy should be performed.