Medullary spongy kidney is a relatively common developmental abnormality of the kidney, mainly manifested by abnormal development of the fine urinary ducts and cystic dilatation in the kidney. Many patients will have a combination of urinary stones, calcification and infection, followed by back pain, hematuria and fever. The development of stones may also be associated with renal tubular acidosis and low citrate levels in the urine. In some patients, stones are continuously discharged from the tiny tubes in the kidney into the main urinary tract, leading to recurrent episodes of back pain, hematuria and other conditions, and may impair kidney function due to repeated combined hydronephrosis, seriously endangering health. Patients with suspected spongy kidneys may find small speckled density shadows in both kidney areas on film. CT scan of the kidneys helps to detect cystic dilatation of fine ducts, small stones, stones draining into the main ducts and hydronephrosis. Treatment is tricky, as the existing technology and conditions cannot completely cure the disease due to the abnormal development of the ducts and renal tubular acidosis, which cannot be completely corrected yet. Treatment is mainly for stones discharged into the main tract, hydronephrosis and infections and other comorbidities. Treatment of stones in the main tract can be done by minimally invasive surgical methods such as extracorporeal shock wave lithotripsy, ureteroscopic lithotripsy and percutaneous nephrolithotomy for stone extraction. Percutaneous nephrolithotomy can also be attempted to remove locally collected stones in patients with frequent stone drainage and localized intrarenal stone collection with significant cystic dilatation of the local tract. Patients without comorbidities such as stone removal can also be observed.