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Ureteral stents are routinely used in urological practice for many indications including obstruction of ureter, ureteral stricture, prior to treatment with extracorporeal shock wave lithotripsy, and to promote healing following ureteral injury. Complications reported with ureteric stents include stent migration, stent rupture, encrustation, ureteral perforation, erosion, and fistulation. Knotting of an indwelling ureteral stent is a very rare complication, with fewer than 30 cases reported in the literature. Techniques for managing this complication include using a holmium laser to cut the knot, percutaneous antegrade removal, and gentle traction. We describe the case of a knotted stent and its removal along with a comprehensive literature review.