By 2000 there have been at least 187 cases of rare pericardial malformations (of them there were 5 cases of the authors) and 1047 cases of celomic pericardial cysts (CPC) (43 cases of the authors) in the literature. Of them 18 (9.6%) patients were found to have no pericardium, the absence of its left half was in 76 (40.6%). No left pericardium was more common in men (61.04%) than in women (38.96%). The literature reports about the absence of the right pericardium only in one case. Partial pericardial defects were encountered in 30.48% of patients with rare pericardial malformations. In general, malformations in the left pericardium were 10 times more common often than those in the right one. The absence of the pericardium or its half most commonly requires no surgical correction. In partial defects, suturing or plastic closure must be performed due to a risk for strangulation and a possible fatal outcome. CPC are not clinically manifested in more than 50% of the patients with CPC, but in some cases dyspnea, dry cough, palpitation may be caused by other causes. Cystectomy yields good results.