Abstract
We report a young female patient with a giant left renal angiomyolipoma (AML) and multiple left retroperitoneal AML. After undergoing robotic-assisted laparoscopic surgery, the tumor was successfully removed cleanly while preserving the normal renal unit. On the fourth day after surgery, she developed respiratory symptoms, including chylothorax, and was diagnosed with sporadic lymphangioleiomyomatosis (LAM) combined with multiple AML. After receiving sirolimus and symptomatic treatment, the patient recovered and had no recurrence of the disease during the follow-up period. This case demonstrates the significant application of robot-assisted laparoscopic nephron-sparing surgery in the treatment of a giant renal angiomyolipoma (14.0 cm in diameter), highlighting its feasibility and effectiveness in managing complex multifocal disease while preserving renal function.