Congenital ascites occurs when there is increased fluid build-up in the peritoneal cavity of the fetus. It can occur in the setting of non-immune hydrops fetalis (NIHF) or be isolated. HF develops when fluid accumulates in extra-vascular areas and cavities of the fetal body. Examples of HF include a pericardial effusion, pleural effusion, ascites, placental growth and uniform skin thickening or skin edema. Congenital ascites can be diagnosed prenatally by an ultrasound exam, through which we are also able to find the cause of some cases of ascites. Biochemical and cytological laboratory analysis have been used in addition to the ultrasound exam, as these methods are more successful in identifying etiologies of congenital ascites. In this review paper, I will discuss several cases of congenital ascites in newborns, which have different etiological origin. In the first case, a neonate presents with congenital chylous ascites and cholelithiasis due to maldevelopment of the lymphatic system. Next, a discussion of four cases will follow in which all neonates present with congenital ascites due to different lysosomal storage diseases. In the third case, a neonate presents with severe congenital ascites and is simultaneously diagnosed with autosomal recessive polycystic kidney disease (ARPKD). The final discussion will focus on a series of 100 cases of congenital ascites for which biochemical and cytological testing were performed to identify the causes of ascites.