Crossed fused renal ectopia (CFRE) is a rare congenital abnormality of the urinary tract where the kidneys are fused on one side, while the ureter of the ectopic kidney crosses the midline with the normal entrance in the bladder on the contralateral side. Congenital anomalies are associated with a stone formation whose management represents a real challenge. To our knowledge, we report the second case of CFRE associated with ureteral stone, which has been successfully resolved with Extracorporeal Shockwave Lithotripsy (ESWL) and the first of its kind where a sufficient degree of stone disintegration has been achieved after a single session with a complete stone clearance during the follow-up. Radiological examination showed an inferior type of CFRE with stone in the proximal part of the ureter of the upper kidney. ESWL is an acceptable and effective treatment option in CFRE patients due to the minimally invasive approach, potentially high stone-free rate, and rare complications.