Renal malrotation is a condition in which the kidney rises from the pelvis to the lumbar region and undergoes a 90° rotation during normal development, causing the renal pelvis to rotate from anterior to medial. Abnormalities in this process can lead to renal malrotation, which is a congenital malformation. The vast majority of these are of no clinical significance and do not require treatment in the absence of complications. During fetal life, the renal germ grows in the pelvis as the fetus grows, and the kidney gradually rises into the renal fossa, with the calyces facing the lateral wall and the renal pelvis opening medially into the midline. Malrotation is a condition in which the embryonic kidney, during the fetal period, stops before reaching its normal direction during its ascent and rotation, resulting in a final misorientation. These patients usually have no special symptoms. If malrotation leads to compression of the renal pelvis and ureter, it may cause complications such as ureteral obstruction and pyelonephrosis, which require treatment. Patients are advised to have regular checkups and maintain good lifestyle habits, such as drinking plenty of water. If there are frequent cases of back pain, stones, infections, etc., or even affect the kidney function, it is recommended to go to the hospital in time and follow the doctor’s instructions for treatment to avoid deterioration of the condition.