The incidence of congenital isolated kidney is about 1/1000 to 1/1500 in newborns, with a male to female ratio of 1.8:1, and renal agenesis is mostly on the left side. Isolated kidney is also called isolated kidney when the diseased side of the kidney has been removed due to kidney disease or when one kidney is non-functional (as determined by intravenous urography, nephrostomy, urine volume and urine specific gravity, nuclear nephrography, etc.). If one kidney is absent, the opposite kidney can usually bear the normal physiological needs of the body, so life is not affected. When isolated kidney and stone, its symptoms and diagnosis are the same as general kidney stone, but when the stone moves down in the kidney, it is more likely to cause obstruction and anuria than double kidney stone, so it is advisable to pay high attention to isolated kidney stone and treat it timely. The choice of treatment for isolated kidney stones should fully protect the kidney function. ESWL is preferred for kidney stones <2 cm in diameter, but it can also be used for acute obstruction. If ESWL is not curative, the interval between the second treatment should be extended appropriately. Holmium laser lithotripsy under ureteroscopy is also an option for less severe fluid accumulation <2 cm in diameter, with more definite efficacy and slightly more expensive cost. ESWL alone is not recommended for stones ≥2 cm in diameter and deerstalker-shaped stones, and percutaneous nephrolithotomy (PCNL) can be used to reduce renal damage, with relatively higher risks. Isolated kidney combined with ureteral stones requires cystoscopic intubation, percutaneous nephrostomy, and holmium laser lithotripsy by ureteroscopy according to the specific conditions such as fluid accumulation and infection.