A 2-month-old, female cat was presented for abdomen dilation. The patient was undernourished, and severe left hydronephrosis was diagnosed after clinical, ultrasonographical and radiographical examination. Although pyelography was performed in order to visualise the ureteral course, surgery was necessary to reach a final aetiological diagnosis and treatment. At gross examination, the left ureter crossed the renal capsula at the level of the caudal renal pole, and the subcapsular ureteral segment was markedly dilated. Distal to the renal capsula, the left ureter was very thin when compared to the right. The parenchyma of the left kidney, as suggested by ultrasonographical evaluation, was extremely reduced in thickness. An ureteronephrectomy was performed. Histopathological evaluation revealed glomerular sclerosis and diffuse parenchymal fibrosis. Severe hydronephrosis derived from an altered renal pelvic anatomy and abnormal ureteral course determining functional stenosis. Diagnosis of congenital anomaly before development of complications such as hydronephrosis could have allowed a surgical renal capsulectomy and obstruction relief. To the author's knowledge, this is the first report of severe hydronephrosis associated to altered renal pelvic anatomy and proximal ureteral ectopia in cat.