Goldston syndrome is a rare condition characterized by cystic renal dysplasia and Dandy-Walker malformation (DWM). We present a case of antenatal ultrasound diagnosis of a fetus with Goldstan syndrome in a 24-year-old primigravida with a history of consanguineous marriage. Ultrasound showed severe oligohydramnios, DWM, bilateral enlarged multicystic dysplastic kidneys and massive fetal ascites. The diagnosis was made at 24 th week of gestation and medical termination of pregnancy was done after discussion with the couple in view of poor prognosis of the condition.