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The clinical spectrum of perinatal infection varies from asymptomatic infection or mild disease to severe systemic involvement. The aim of this paper is to present a severe intrauterine infection, which led to difficulties in diagnosis and unfavorable evolution., Case Presentation: M.E., 6-weeks-old, born small for gestational age, was admitted in our Hospital for gastrointestinal signs: diarrhea, abdominal distension, observed three days earlier. Clinical and biological exams revealed hepatic disease related with hepatic cytolysis and cholestasis. Abdominal ultrasound showed large amounts of ascitic fluid, cirrhotic liver, enlarged portal vessel with hepatopetal flow, normal gallbladder and biliary tract. Computed tomography (CT) angiography revealed a wide hepatic artery, the presence of portal vein and absence of splenic vein. Serology detected IgM anti-cytomegalovirus antibodies. Postmortem histological exam confirmed the liver cirrhosis; cell free cytomegalovirus (CMV) antigens were found among alveoli with atelectasis., Discussion: The onset of hepatic disease was acute or chronic? Anamnesis offered reliable diagnostic criteria for intrauterine infection (flu during first trimester of pregnancy, intrauterine growth restriction, prolonged jaundice). The mother had been tested for all TORCH (toxoplasmosis, other agents, rubella, cytomegalovirus, herpes simplex) infections other than CMV. The strict liver cell tropism raised questions regarding virulence, host immunology or the existence of other disease (vascular?) Conclusions: Liver cirrhosis at this age is very rare; the most frequent etiology is viral. The late diagnosis of this case led to the impossibility of etiological treatment.