A 38-year-old Latin-American man developed uremic syndrome without any
evidence of previous kidney diseases or any other health problems. Ul
trasound and CT scan confirmed normal size of the kidneys without evid
ence of urinary tract obstruction or renal parenchymal cysts. Kidney b
iopsy showed cystic dilatation of Bowman's space (glomerulocystic kidn
ey disease). The patient was started on hemodialysis. Severe renal dys
function and uremic symptoms are a rare initial manifestation of glome
rulocystic kidney disease. This pathology should be considered in the
differential diagnosis of patients with normal size kidney and chronic
renal failure.