In nephrology practice, the association of renal failure and deafness immediately brings to mind the Alport syndrome. However, in the differential diagnosis of deafness and renal failure a great number of syndromes ranging from Alport to Muckle-Wells, Refsum disease, Cockayne syndrome, familial renal tubular acidosis, Charcot-Marie-Tooth, Alstrom, mitochodrial disorders and hypo/hyperparathyroidism should be thought. With this case, we wanted to remind the clinician these syndromes leading to deafness and renal failure. At the same time, the association of double ureters and Alport syndrome was described for the first time in literature.