Systemic hypertension (HTN) is one of the major problems associated with chronic renal failure (CRF). HTN is a symptom and complication of CRF. The prevalence of HTN varies with the cause of CRF. The incidence of HTN increased up to 90% with progressive deterioration of the glomerular filtration rate (GFR). HTN is the major risk factor for decline in renal function and progression of CRF. It is the most important risk factor for cardiovascular diseases and morbidity and mortality in patients with CRF and end-stage renal disease (ESRD) on dialysis. The target blood pressure for hypertensive children with CRF should be under the 95th percentile for sex and age. The therapeutic approach in CRF is directed at reducing volume expansion and sodium retention, and decreasing peripheral vascular resistance. Diuretics are first-line therapy for HTN in patients with CRF with sodium and water retention. ACE inhibitors are the first-class drugs because of their renoprotective effect in preventing deterioration of kidney function. Calcium channel blockers are excellent first-line antihypertensive drugs. Recently angiotensin II receptor blockers and ACE inhibitors have been efficiently used together for the treatment of HTN and to prevent further decline in renal function.