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Abstract Congenital and hereditary cystic lesions of the abdomen are relatively rare. Correct diagnosis is critical as they may simulate several other benign and malignant acquired diseases of the abdomen. With the correct and appropriate use of imaging, diagnosis may be relatively straightforward and clinical management may be implemented appropriately. The purpose of this article is to describe imaging findings of common and uncommon congenital and hereditary cystic disease of the abdominal organs.