Unilateral and segmental localised polycystic disease is a rare type of cystic disease of the kidney. It takes the form of a segmental cystic abnormality in one kidney morphologically identical to the autosomal dominant adult form of polycystic kidney disease. The clinical, radiological, and pathological appearances of a case are described. The diVerential diagnosis and a possible pathogenic mechanism are discussed. (J Clin Pathol 1998;51:703-705) Keywords: kidney; polycystic disease; localised polycystic disease Case report A 45 year old male presented with left abdominal and loin pain associated with sterile pyuria. Tenderness in the left upper abdominal quadrant was elicited by clinical examination. An abdominal ultrasound showed a multicystic mass at the lower pole of the right kidney, the Clin Pathol 1998;51:703-705 703