A patient with eight years' history of haemodialysis treatment developed gross haematuria. During evaluation severe abdominal pain with dyspnoea developed and he died suddenly. Autopsy revealed spindle cell type renal cell carcinoma (RCC) associated with acquired renal cystic disease and adenoma in the remaining kidney. Multiple embolization of RCC was considered to be linked to the cause of death. Although patients on long-term haemodialysis often have multiple renal cystic lesions, some of which are associated with tumour formation, RCC of this specific cell type in dialysed kidney has not been previously described.