Nevoid basal cell carcinoma syndrome (NBCCS) is a genetic disorder known for causing skin cancers and jaw cysts, but it has a wide breadth of additional manifestations across multiple organ systems, which may go unrecognized. This study explores the link between NBCCS and urinary tract anomalies by examining affected patients at a single center, where cases of ureteropelvic obstruction and renal agenesis were identified. A review of the medical literature further revealed urinary tract anomalies in NBCCS cases, highlighting this as a variable feature associated with the syndrome, which may be investigated with renal ultrasound at the time of diagnosis. (By Dr Nicholas A Borja, https://jmg.bmj.com/content/early/2024/12/31/jmg-2024-110340 )