A ureteral rhabdomyosarcoma was diagnosed in a 2-year, 9-month-old, entire, male dobermann with persistent haematuria. Diagnostic imaging revealed unilateral hydronephrosis and hydroureter. In addition, it showed thickening of the distal ureteric wall and focal thickening of the mucosa of the bladder trigone between the ureters. No mass was identified. The patient underwent exploratory laparotomy, and biopsies of the bladder mucosal thickening and right ureteral thickening were obtained. Histopathology of the ureteral thickening was suggestive of a ureteral rhabdomyosarcoma. A right ureteroneocystostomy was performed, and histopathology confirmed the suspected ureteral rhabdomyosarcoma, which was fully excised. Six months later, the patient remains asymptomatic with no evidence of recurrence or metastases on abdominal ultrasonography.