Abstract:Background: Congenital anomalies of the abdominal wall are classifi ed as anomalies with the abdominal wall defect (omphalocele, gastroschisis) and without the defect (umbilical hernia, persistent ductus omphaloentericus or urachus). Clinical presentations of these conditions are different, and so is the timing of surgical intervention and approach with or without the exploration of the peritoneal cavity. Case: The presented case report refers to a rare fi nding of ectopic liver forming mesodermal cyst within the umbilical region. Full term neonate girl with 5 cm spheroid tumor in umbilicus was otherwise without problems. Ultrasonography (USG) of the lesion detected a tissue with good vascularization and a cystic cavity. There was no fl ow in the umbilical vessels and no evidence of intestinal loop in the sac. USG of the liver was normal. During surgery an additional narrow canaliculus was identifi ed connecting the spheroid along with umbilical vessels to the liver. The whole spheroid together with the canaliculus was surgically removed without exploration of the abdominal cavity. Histological evaluation of the surgical specimen discovered liver tissue with a mesodermal cyst in the center. The sphere was connected to the liver by a bile duct. Six months after the operation the child is in a good clinical condition. Conclusion: In conclusion omphalocele may contain liver. Ectopic liver is an extremely rare condition. Surgical treatment in the presented case focused only on umbilicus without exploration of the abdominal cavity and appeared to be suffi cient. Long-term postoperative follow up typical in pediatrics will be applied also in this patient (Fig. 3, Ref. 25). Full Text in PDF www.elis.sk.