| Summary: | This case report is intended to describe a rare case of splenic infarction (SI) associated with biliary atresia, which contributed to the worsening of the prognosis. In this case study, a boy diagnosed with biliary atresia underwent the Kasai procedure at the age of 3 months. The patient presented an intense abdominal pain, ascites and fever at the age of 6 years. A computed tomography (CT) scan showed enlargement of the spleen and an image suggestive of SI. The symptoms improved with the administration of antibiotics, analgesics, while ascites decreased with the use of diuretics. Liver transplantation was indicated. SI is an extremely rare event characterized by vessel occlusion, parenchymal ischemia and tissue necrosis. The symptoms are non-specific and may be confounding factors with other causes of acute abdominal pain. Splenomegaly-inducing diseases may be a risk factor. There are few case reports of SI and cirrhosis of different etiologies in childhood. Abdominal CT scan with intravenous contrast is the imaging modality of choice and the treatment ranges from supportive care to splenectomy. Despite being a rare condition, its knowledge is extremely important, as it allows early clinical suspicion, with immediate institution of treatment, which may interfere with the prognosis.
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