Unilateral and segmental localised polycystic disease is a rare type o
f cystic disease of the kidney. it takes the form of a segmental cysti
c abnormality in one kidney morphologically identical to the autosomal
dominant adult form of polycystic kidney disease. The clinical, radio
logical, and pathological appearances of a case are described. The dif
ferential diagnosis and a possible pathogenic mechanism are discussed.