We evaluated the efficacy of nephrectomy for the management of nephrogenic hypertension in children. We retrospectively reviewed the records of 320 children who underwent nephrectomy between 1991 and 2001, and 22 underwent nephrectomy for the management of hypertension. Of the 22 patients 16 (73%) had long-term followup, including 8 with end stage renal disease who were dialysis dependent, and 8 with normal renal function and unilateral parenchymal renal disease (UPRD). Within the UPRD group 3 patients had renovascular hypertension, 3 had reflux nephropathy, 1 had renal artery thrombosis and 1 had Page kidney. Age at hypertension onset, age at nephrectomy and elapsed time between diagnosis and intervention were studied. Antihypertensive medication requirements before surgery and after postoperative followup were evaluated to assess treatment efficacy. Complete success was defined as blood pressure normalization without antihypertensive requirements. Partial success was defined as decrease in medication requirements and/or discontinuation of minoxidil. Failure of treatment was defined as persistent hypertension, increased medication requirements or minoxidil dependence. In the end stage renal disease group mean age at diagnosis was 5.9 years (range 15 months to 10 years) and bilateral nephrectomy was performed at a mean age of 8.9 years (19 months to 15 years) with average elapsed time between diagnosis of hypertension and nephrectomy of 3 years. After a mean followup of 4.4 years (range 6 months to 8 years) 7 patients (88%) experienced complete or partial success and nephrectomy management failed in 1. In the UPRD group average elapsed time was 2.2 years (range 1 month to 10 years) between a mean age at diagnosis of 6.7 years (birth to 16 years) and a mean age at nephrectomy of 8.9 years (1 month to 17 years). After a mean followup of 1.6 years (range 1 month to 5 years) complete or partial success was experienced by all 8 patients (100%). All 8 UPRD group patients experienced adequate residual renal function. The vast majority of patients in both categories experienced complete or partial success from nephrectomy for the management of medication refractory hypertension. Nephrectomy for hypertension control is safe and effective, and obviates the need for morbid medications. We continue to accrue patients in a prospective manner.