AGT and renal tubular dysgenesis of genetic origin: The primary pathophysiology in RTD is reduced production and activity of angiotensin II (ATII), which occurs because of genetic mutations in the renin-angiotensin system and decreased release of aldosterone and vasopressin [2-4]. Clinicians should be vigilant for potentially life-threatening hypotension during anesthesia in these patients, given that RTD carries substantial anesthetic risks such as pronounced vasodilation and severe hypotension. However, there is a lack of evidence on anesthesia management in RTD, which can be explained by the rarity of survival beyond the neonatal period.