“Pediatric renovascular hypertension (PRH)” is a rare but potentially dangerous syndrome that, if not identified and treated right away, can result in problems like chronic renal disease and cardiovascular disease. The pathogenesis, clinical manifestation, diagnosis, and therapy of PRH are discussed in this review, with an emphasis on evidence-based methods for treating this condition. PRH has a complicated etiology that includes a number of variables, including renal artery stenosis, RAAS activation, and oxidative stress. Depending on the underlying cause of the hypertension, the clinical presentation may differ, but it may include signs and symptoms like headaches, exhaustion, and breathing problems. Imaging tests like renal Doppler ultrasound and magnetic resonance angiography may be part of the diagnostic evaluation. Antihypertensive drugs, revascularization techniques, and lifestyle changes are all available as treatments. Maintaining blood pressure management and identifying issues early require close monitoring and follow-up. To manage juvenile renovascular hypertension as effectively as possible, pediatricians, nephrologists, radiologists, and surgeons must work together.