The authors report a rate case of carcinoid syndrome (CS) in a 12-year-old child that was caused by a carcinoid tumor located in the ileum that had metastasized mainly to the liver. After resection of the primary tumor, treatment with octreotide and polychemotherapy (five monthly cycles of 5-flourouracil, epidoxorubicin, and deticene) was ineffective for both reducing the metastatic liver disease and controlling the clinical symptoms. The patient's poor prognosis led the authors to perform a liver transplantation. The results of liver transplantation in patients with metastatic liver disease are generally not good. However, neuroendocrine tumors seem to be an exception due to their slow growth. At present, the child is in complete clinical and laboratory remission 4 months after transplantation. To the best of our knowledge, only 5 cases of CS have been reported to date in children.