(UroToday.com) The 2021 American Urological Association (AUA) Summer School session on Upper Tract Urothelial Carcinoma included a case-based discussion led by moderator Dr. Surena Matin who was joined by panelists Dr. Sima Porten and Dr. Vitaly Margulis. This case highlighted the diagnosis and treatment of upper tract urothelial carcinoma in patients with suspicion for hereditary disease. This patient was a 56-year-old female with gross hematuria and flank pain with no medical history and a surgical history consistent with a hysterectomy in 1998 and two C-sections. Her family history was negative for genitourinary malignancy, however, her father, sister, and paternal uncle had colon cancer and her father had skin cancer. Physical examination was unremarkable, however, a CT scan demonstrated a 3 cm right distal ureteral mass with hydronephrosis. Cystoscopy and right ureteroscopy showed no bladder tumors, but a bulky low-grade appearing tumor in the distal ureter and no tumors in the proximal ureter and renal pelvis: