Goldston syndrome is a rare entity describing the association of polycystic kidneys and Dandy Walker malformation with or without hepatic fibrosis. A 28 years old pregnant woman came to radiology department for the first antenatal scan. Ultrasound showed a single fetus corresponding to 27 week of gestation. The fetal brain revealed deficient vermis with a posterior fossa cyst communicating with the 4th ventricle suggestive of Dandy-Walker malformation (DWM). The kidneys were enlarged, and echogenic containing small cysts with associated amniotic fluid index of 5 cm suggestive of oligohydramnios. A diagnosis of Goldston syndrome was suggested. These findings were confirmed on post natal head and renal ultrasound.