A 6-year-old female child sought medical service due to a gastrointestinal malformation. During medical follow-up, partial absence of the inferior vena cava was diagnosed, a rare congenital alteration affecting the vascular drainage from the inferior segment of the body. Imaging exams were accomplished, contributing to evaluation and description of the case. Conservative treatment with oral anticoagulant was maintained. The patient presents good evolution after long-term cardiovascular follow-up.
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