A 14-year-old Swiss Warmblood gelding was presented with chronic severe polyuria, polydipsia and weight loss. At the time of admission, water intake was 240 l/day. On rectal examination, a large mass was identified in the left dorsal abdominal quadrant, which was shown to originate from the left kidney by transabdominal ultrasonographic examination. Unilateral nephrectomy via flank incision was performed under general anaesthesia. Histopathological examination of the tumour revealed a papillary renal adenocarcinoma. Successful outcome and survival was documented 13 months after surgery. Severe polyuria and polydipsia should be considered as major clinical signs for renal carcinoma in horses, which can be successfully treated with unilateral nephrectomy if no signs of metastatic spread are evident.