Congenital leukemia, which occurs before 4 weeks of life, is distinguished from that occurring in childhood. It is often associated with MLL gene rearrangement and its prognosis is poor. Chemotherapy alone does not yield a good survival chance, so stem cell transplantation is often tried nowadays. Congenital leukemia occurring on the first day of life is rarely reported. We report a female term infant delivered by Cesarean section who presented immediately after birth with tachypnea, purpuric rash, subcutaneous nodule (leukemia cutis), absence of Moro reflex, and extreme leukocytosis. The patient was diagnosed as acute lymphocytic leukemia with t(11;19)(q23;p13) by peripheral blood analysis and died of the disease 10 hours after birth.