The case of a newborn male infant with congenital Langerhans cell histiocytosis (LCH) is presented. At birth, showed cutaneous lesions (papules and vesicles with a haemorrhagic aspect), mucosal and ganglionic involvement. Biopsy of these lesions led to the diagnosis of LCH. At 24 hours of life the patient began with respiratory, hepatic, hematological and renal dysfunction, and died at 72 hours of life, despite corticoid treatment. LHC with vesicles and a rapid and fatal development, has previously only been described in three patients. The differential diagnosis of a disseminated and hemorrhagic vesicular eruption in a newborn infant is extensive and must include LHC.