Ten patients with congenital defects of the pericardium were treated in Departments of Cardiac Surgery, Silesian School of Medicine in Zabrze and Katowice between 1989 and 1998. There were eight children and two adults, eight males and two females. In each case the pericardial defect was discovered intraoperatively during surgery for congenital heart defect. There were no cases with clinical symptoms that could be clearly related to the defect of the pericardial sac. In the case of a child with a complete absence of the left pericardial wall the heart was significantly rotated contrary to the defect. The final outcome of the congenital heart defect surgery was satisfactory in each case. An abbreviated historical review of the diagnosis and treatment of the pericardial defects is presented with special attention placed on therapeutic management. Surgical correction of pericardial defects is concluded to be justified in patients with clinical symptoms. In most cases pericardial defects are discovered intraoperatively, but when they are large the said defects do not require any treatment.