A 17 year old female with diabetes mellitus type 1, with chronic kidney disease. Moreover, hypoplastic left kidney and a renal mass on the functional kidney. The possibility all these medical problems occurring at the same time is close to impossible. The management is likewise very difficult and challenging. Partial nephrectomy as initial choice to treatment but initial ureteroscopic findings and change to radical nephrectomy was prudent. In lieu of chronic dialysis, the decision of early transplantation was done. We present the management choices we had to make in a very difficult case.