We present a 55 year old male who was investigated for painless macroscopic haematuria and had essentially normal radiological and cystoscopic findings. He progressed rapidly and was eventually diagnosed with a Collecting Duct Carcinoma. This case is of interest as it is the first reported case of Collecting Duct Carcinoma occurring bilaterally. It is also the first case to cause end stage renal failure requiring dialysis due to extensive tubular involvement. Finally, it is the first time this malignancy has been found to cause a tumour associated nephritis. We describe the clinical course and present the various histological findings followed by a review of the literature.