Background Primary ureteral neoplasia in dogs is extremely rare. To the best of the authors’ knowledge, this is the second documented case of a primary ureteral hemangiosarcoma. This case report describes the clinical and pathological findings of a primary distal ureteral hemangiosarcoma. Case presentation A 12-year-old spayed female goldendoodle was presented with a history of polyuria and weight loss. Abdominal radiographs revealed a large cranial abdominal mass. Abdominal ultrasound and computed tomography (CT) identified a left sided distal ureteral mass with secondary hydroureter and a left lateral hepatic mass with no evidence of connection or diffuse metastasis. A left ureteronephrectomy, partial cystectomy, and left lateral liver lobectomy were performed. Histopathology was consistent with primary ureteral hemangiosarcoma and a hepatocellular carcinoma. Adjunctive therapy including chemotherapy was discussed but declined. Conclusion Due to its rarity, the authors of this case presentation believe that ureteral hemangiosarcoma should be included as a differential diagnosis when evaluating a ureteral mass. With the unknown, and suspected poor prognosis, routine monitoring with adjunctive therapy should be considered.

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