Polyhydramnios and oligohydramnios in twin gestation is most often caused by twin-twin transfusion syndrome. Presented is a monozygotic twin pair with polyhydramnios and oligohydramnios, in which both twins had glomerulocystic kidney disease of differing severity. The more severely affected donor twin died of renal failure in the neonatal period. The surviving twin is well following unilateral nephrectomy. This case illustrates the varied spectrum of pathology in glomerulocystic kidney disease.
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