Disturbances of the normal asymmetric placement of organs, such as situs ambiguous or situs inversus, have been defined as laterality defects. The phenotypes of which included the association of laterality defects with renal-hepatic-pancreatic dysplasia, and with midline anomalies were reported. Defects of laterality frequently are associated with midline anomalies, which both result from a disturbance of pattern formation during blastogenesis. The combination of dysplastic kidney, pancreas and liver or autosomal recessive polycystic kidney and hepatic fibrosis could be explained by a common error of histogenesis. We presented the case of situs inversus totalis, polycystic kidney disease, hepatic fibrosis, and occipital encephalocel who diagnosed and terminated in 15.th weeks of gestation antenatally. These disorders are a group of anomalies affecting fetus in similar embryological periods and arising from similar gene defects. In the presence of one of the disorders, the others should be investigated. Most of the disorders are manifested by cystic kidney and oligohydramnios, the pathology may be diagnosed by prenatal ultrasound examination.

Keywords: Situs inversus, polycystic kidney disease, encephalocele, hepatic fibrosis