Many complications due to double j (DJ) stent placement have been reported. DJ stent knotting is a rare complication, with only a few cases reported in the literature. We presented a case of DJ stent knotting and reviewed the literature regarding this complication. We reported a 20-year-old man with a history of cystinuria and ureteral stone managed with retrograde ureteroscopy and holmium laser three months ago. The patient comes for DJ stent removal. Firstly, we tried to remove the DJ stent via the cystoscopic procedure, which failed. A fluoroscopic image revealed a knotted DJ stent lodged at the ureteropelvic junction and was removed via holmium laser ureteroscopic procedure without complications. In conclusion, when cystoscopic procedure with simple traction fails to remove DJ stents, multimodality urological procedures such as holmium laser should be tried, especially in patients with urolithiasis predisposing factors.