Inverted papillomas are benign urothelial tumors. The majority of cases occur in the bladder, while inverted papillomas of the upper urinary tract are very rare. The association of inverted papillomas of the bladder and urothelial malignancy is documented, but the incidence is low. Inverted papillomas of the upper urothelial tract coexist frequently with urothelial malignancy. Close follow-up with urography and cystoscopy is mandatory in such patients. We describe a case with synchronous bilateral ureteral inverted papilloma and a transitional cell tumor of the bladder, illustrating the strong association of upper urothelial tract inverted papilloma and urothelial malignancy.