Arterial hypertension in children up to 6 years of age is mostly secondary, and renal parenchymal disease is the leading cause of hypertension in children. Coarctation of the aorta is a rare cause and should be always excluded. A 6-year-old girl was referred to pediatric nephrology consultation for enuresis, high blood pressure, recurrent night headache, and sporadic pain in the lower extremities. On examination, a systolic murmur was detected, radiating to the carotids and the back, and the patient had weak femoral pulses as well. The echocardiogram confirmed a severe aortic coarctation. Surgical correction was performed and resulted in hypertension improvement and resolution of symptoms. Coarctation of the aorta may not be diagnosed in the neonatal period. The present case report highlights the importance of blood pressure monitoring in children and the need for a careful investigation of the secondary causes of hypertension and an early diagnosis of coarctation of the aorta for a timely surgical correction that improves prognosis.