Primary liver tumors in children are infrequent, accounting for 0.5-2 % of all pediatric neoplasms. About two-thirds are malignant. The most common pediatric malignant liver tumours are, in order of frequency, hepatoblastoma, hepatocellular carcinoma, and embryonic sarcoma of the liver. Among benign tumors, the most frequent are hemangioendothelioma and mesenchymal hamartoma of the liver, and more rarely focal nodular hyperplasia and hepatocellular adenoma [1,2].

We report the case of an infant who was entrusted to us for the management of a large liver tumor. Radiology (CT scan and MRI), in addition to the elements in favor of a mesenchymal hamartoma of the right liver revealed a congenital anomaly associated, in this case, with the absence of the inferior vena cava in its intraabdominal portion. A right hepatectomy enlarged to segment IV was performed.

The right renal vein drained into the right suprahepatic vein was preserved. Note that this tumor represents 50% of the total weight of the patient, which explains the difficulties and constraints that hindered the development and quality of life of the young patient.