Autosomal dominant polycystic kidney disease with congenital absence of contralateral kidney

Autosomal dominant polycystic kidney disease is the most frequent hereditary kidney disorder, accounting for 8-10% of the cases of end-stage renal disease. It is characterized by bilateral multiple renal cysts, nevertheless, asymmetric enlargement of the kidneys is frequently observed, and this can lead to diagnostic confusion. We report the rare occurrence of autosomal dominant polycystic disease confined to a right kidney and congenital absence of the contralateral one. Unexpected early onset of terminal renal failure in this hypertensive 23-year-old male is discussed with the review of the literature.