Renal tubular dysgenesis is a rare disorder of differentiation of the fetal kidney. The condition has previously been reported as a postmortem diagnosis in infants who have had oligohydramnios commencing after 20 weeks gestation and have died of renal or respiratory failure shortly after birth with a clinical description of Potter sequence. The absence of clinically significant pulmonary hypoplasia in our case serves to emphasize that renal tubular dysgenesis, fetal anuria and long-standing oligohydramnios can occur without pulmonary insufficiency. The coexistence of renal tubular dysgenesis with neonatal hemochromatosis has been previously described in four published cases. The link between these two rare conditions is clinically important if dialysis or liver transplantation is considered in infants with hepatic and renal failure. Antemortem diagnosis by renal biopsy in our case enabled parental counseling and avoided the inappropriate use of peritoneal dialysis.