A clinical case of a 50 year old male, diagnosed in 1984 as having renal polycystosis is presented; four years later, due to the availability of complementary exploratory methods with better resolution and a detailed examination of the patient, the presence of tuberous sclerosis with multiple organ involvement was confirmed. It is noteworthy his florid renal pathology with multiple angiomyolipomes and cysts associated to lithiasis, on a horseshoe kidney. It was this fact that encouraged us to publish the case due to its originality.