Immunoglobulin G4-related renal disease masquerading as renal pelvic tumor

The recently recognized immunoglobulin G4 (IgG4)-related disease presenting as renal pelvic pseudotumor is rare. A definitive diagnosis is often difficult to obtain preoperatively, with patients being subjected to radical surgery due to suspicion of malignancy. We present a 64-year-old male with lower urinary tract symptoms, who, on evaluation had a right renal pelvic tumor on imaging and ureteroscopy. The patient underwent laparoscopic radical nephroureterectomy on clinical suspicion of upper tract urothelial carcinoma. The final histopathology revealed IgG4-related disease.