The most common variant of renal cell carcinoma in ESRD patients is papillary renal cell carcinoma .(1,2) Squamous cell carcinoma of the renal pelvis is a very rare variant, its incidence range from less than 1 percent to less than 5-10 percent of all the upper urothelial tract tumors. (3,4) Squamous cell carcinoma in ESRD is mentioned in the literature very few, There is no definite cause to blame, but smoking, recurrent operation as PCNL, chronic irritation caused by stones or infection and squamous metaplasia are risk factors. (5–7) The most common cause is stones leading to squamous metaplasia, which was found in the previous kidney specimen that was removed from the patient two years ago. Squamous metaplasia considered by some authors as precancerous lesion, occurs more in the lower tract unlike the upper tract, and requires strict follow up. (5,8) The clinical presentation of the disease includes no characteristic symptoms and signs, making it very difficult to diagnose as in this case the diagnosis was difficult, all the signs were making the diagnosis directed toward infection or retro-peritoneal abscess also the CT guided biopsy was not conclusive, until exploration was done.(9) Pain, hematuria, local swelling of the loin and a fistula formation or skin sinus as in our patient are symptoms that may occur in advanced disease (5,7,10) . The treatment is radical surgery, due to the rarity of the cases the conclusive decision of applying radiotherapy or chemotherapy either in advanced or early cases, preoperatively or postoperatively, can’t be taken. (10–12) To achieve similar outcome as transitional cell carcinoma the diagnosis must be early, as the main problem with this variant is its delayed diagnosis either according to the clinical picture or radiological(13).