Nonimmune hydrops fetalis is becoming a predominant form of fetal hydrops due to the declining incidence of immune hydrops fetalis triggered by Rh isoimmunization. Infantile polycystic kidney appeared to be related to hydrops fetalis whether it is causal or merely coincidental and may represent another entry to differential diagnoses. Infantile polycystic kidney was diagnosed by an elevated maternal serum alpha-fetoprotein (AFP) value coupled with an ultrasonographic abnormality scanned as a multicystic mass with ascites in the fetal abdomen antenatally. This study presents a case of infantile polycystic kidney that resulted in a stillborn baby with hydrops fetalis and extensive placental calcification; it was the first case in Korea in which nonimmune hydrops fetalis was associated with infantile polycystic kidney in consecutive siblings by autosomal recessive inheritance in one family. In addition, this paper comprehensively reviews the incidence, etiology, prenatal diagnosis and proper management of nonimmune hydrops fetalis.