We report a patient with autosomal dominant polycystic kidney disease
receiving hemodialysis who developed a renal leiomyosarcoma with retro
peritoneal spread and intestinal involvement. We discuss the different
ial diagnosis of tumours and neoplasms in polycystic kidneys. We highl
ight the patient without symptoms or signs and the importance of the a
bdominal scan. We constrast the rarity of sarcoma with the much common
er renal carcinoma in autosomal dominant polycystic renal disease.