The administration of angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin type 1 receptor blockers (ARBs) to pregnant women has been reported to cause ACEI/ARB fetopathy, including oligohydramios, pulmonary hypoplasia, renal insufficiency, limb contracture, and fetal hypotension in the child. Most of the patients die or develop end-stage renal failure during the neonatal period. The long-term prognosis of renal dysfunctions of patients with ARB fetopathy has not been reported. We report two pediatric cases, a 6- and 2-year-old boy, respectively, with ARB fetopathy whose renal functions were thoroughly evaluated after recovery from neonatal renal failure. Both patients showed (1) mildly decreased glomerular filtration rate, (2) no significant proximal tubular dysfunctions, and (3) salt-losing nephrogenic diabetes insipidus, while the excretion of arginine vasopressin and urine level of cyclic AMP were increased. The data on these two patients indicate that the administration of ARB to the fetus profoundly impairs the urine concentrating ability, probably due to papillary atrophy and the disturbed formation of the osmotic gradient in the medulla, which have been confirmed in neonatal rats administered with ACEIs or ARBs. ACEIs/ARBs must not be administered to pregnant women.