Glomerulocysüc kidney disease (GCKD) is an unusual form of cystic renal diseases in childhood characterized pathologically by cystic dilatations in Bowman's space. GCKD may be relatively underreported in children with end-stage renal disease (ESRD) because definitive diagnostic testing with a renal biopsy is not routinely performed in patiens with ESRD. We present a case of GCKD in a 5-year-old girl who presented with ESRD and underwent chronic peritoneal dialysis. Additional findings in this patient were cerebral atrophy, mental retardation and hypermetropia that has previosly been unreported in patients with this disease. The clinical features of this rare condition and the diagnostic approach are reviewed. In conclusion, although rare, GCKD should be considered in the differential diagnosis of ESRD in childhood.