Introduction: Development of renal cell carcinoma (RCC) is a life threatening complication of long- term haemodialysis. Studies have shown that approximately one third or more of patients on long-term haemodialysis of more than three years will develop acquired cystic kidney disease (ACKD), and 20- 50% of patients with ACKD, particularly with complex cysts of Bosniak types IIF, III and IV, may even- tually transform into RCC. This study looks at the incidence of haemodialysis related RCC in Brunei Da- russalam. Material and Methods: Cases of RCC registered in the Department of Pathology registry from January 2000 to December 2010 were identified and retrospectively reviewed. Results: A total number of 5,287 malignancies were reported during this period of which 74 were RCC, giving an overall incidence of 1.4%. Out of these 74 cases, 6.8% (n=5) was found to be RCC transformed from ACKD associated with long-term haemodialysis. There was a male preponderance (4:1), four Malay men and one Chinese lady with a median age of 51 years (37-66) at diagnosis. The duration of haemodialysis prior to the development of RCC ranged from 4 to 16 years. The common presenting signs and symp- toms were cystic mass in kidney 60% (n=3), haematuria and metastatic RCC in urinary bladder 20% (n=1), metastasis to the neck glands 20% (n=1). The right kidney was affected in four (80%) and one on the left (20%). Conclusion: Our study showed that ACKD associated RCC accounted for 6.8% of all RCC, and the duration to RCC development ranged from four to 16 years. There was a preponderance in males. Radiological screening may need to be considered for patients at risk.