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The authors presented a rare condition of cystic lymphangion of the liver in a female child of 14 months, comprising the whole left lobe leading to compression of the surrounding intestinal organs and partly of some chest organs. By a transversal supraumbilical laparotomy, the cystic tumor of the left liver lobe was identified, and a left lobectomy of the liver was made by removing all cystic formations. It was hystologically acknowledged that it was about a cystic lymphangiom of the liver. Postoperatively the course ran regularly and with no complications. The control examinations showed that the function of the liver was not damaged. The cystic changes of the liver are most frequently in the framework of the polycystic diseases when even other organs are invaded. All cysts of the liver can be divided into congenital cysts or acquired. The presented case was most probably about a congenital anomaly, which was getting augmented gradually and resulted to a compression of the surrounding organs.