摘要:Polycystic Kidney Disease (PKD) is an autosomal recessive disease with an incidence of about 1 in 30 000 births. It characterized by multiple cysts which filled by fluid that can ultimately impede kidney function leading to degeneration of renal tissue and renal failure. Oligo or anhydramnios is frequently present but not invariably so, suggesting that some degree of renal function is retained in some PKD cases. We present a 30 year old woman, gravida 5, para 4, at 22 weeks of gestation with ultrasound findings of autosomal recessive PKD, unilateral ventriculomegaly and marked oligohydramnios. Ventriculomegaly is a brain condition that occurs when the width of the atrium of the lateral ventricle is greater than 10 mm and occurs in 0.3-1.5 births per 1000. The association between autosomal recessive PKD and unilateral ventriculomegaly is not well understood and needs further evaluation.