Leukemia is not uncommon disease of infant and children. The incidence in 0- to 4-year old range is estimated at 5 per 100,000 and the over all incidence at 1 per 15,000. However, since acute leukemia in newborn infants extremely rare and also because of its dramatic appearance and the unexpected sudden death, we have presented a case of congenital acute lymphocytic leukemia in an infant of 45 days of age. The infant was apparently well until forty fifth day of life when he suffered from symptom of URI with fever and noticed abdominal huge palpable mass. At this time, total white blood cell count was highly elevated, 198,000 per cu mm(lymphocyte : 72%, segmented neutrophil : 3%, myelocyte : 2%, prolymphocyte: 12%, lymphoblast : 11%). Hemoglobin was 6.3 g/dl, hematocrit was 20%, and bone marrow finding revealed mainly blast form with immature lymphocytes. Finally, we diagnosed the case as congenital acute lymphocytic leukemia. A review of literature briefly was done.