Renal Tubular Dysgenesis (RTD) is a rare, autosomally transmitted, congenital abnormality of renal development, characterized by abnormal maturation or differentiation of the renal tubules, especially the proximal ones. The condition is associated with persistent fetal anuria, early-onset oligohydramnios, skull ossification defects, preterm birth, and Potter's Syndrome.

Diagnosis is based on the morphologic analysis of the kidneys, and is confirmed by a histological finding of abnormalities in the proximal tubules; specifically the poor development or shortening of these tubules. Prognosis of the condition is extremely poor. Most affected patients develop renal insufficiency and die within a few days of birth. There have been only a couple of reported cases of patients surviving the neonatal period.

The inherited form of RTD is transmitted in an autosomal recessive form. Mutations in several genes encoding the components of the renin-angiotensin system have been shown to result in RTD. These genes include those that code for Renin, Angiotensinogen, Angiotensin Converting Enzyme, and the Angiotensin II Receptor Type I.