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Objective. The objective of the present report was to describe clinical, ultrasonographic, and surgical findings, as well as the therapeutic choice, of a rare case of canine ureteral torsion associated with hydronephrosis and unilateral ureterolithiasis. Patient evaluation. A 9-year-old female dog was seen with a history of swelling in the ear region and a tumor in the mammary gland number five of the right chain. In the ultrasound examination for tumor staging, abnormalities were seen in the kidney and left ureter. Pelvic and ureteral dilation, sediment, and diverticular structure in the ureter were abnormal findings. The clinical manifestations were not specific to the urinary tract and were not suggestive of the observed ultrasound abnormalities. An exploratory laparotomy was performed and confirmed the suspected ultrasound diagnosis. In addition, it was possible to identify two twisted ureteral segments. Left unilateral nephrectomy was performed to correct the ureterolithiasis and ureteral torsion. At 15 days postoperatively, no urinary or laboratory changes were identified. Conclusions. Because it was possible to verify the abnormalities in the left ureter and kidney before the development of clinical signs, it was possible to institute the appropriate therapy promptly and prevent the disease progression. Keywords: Stenosis; canine; urinary; kidney; obstruction https://doi.org/10.21897/rmvz.2397