Type I tyrosinemia (HTI) is an autosomally recessively inherited disease caused by deficiency of fumarylacetoacetate hydrolase. The disease manifests with liver failure, renal tubular defects, and neurologic crises. Currently orthotopic liver transplantation (OLT) enables patients to survive. However, renal fumarylacetoacetate hydrolase deficiency is not corrected by OLT, and the long-term prognosis of the nephropathy is not known. We investigated tyrosine metabolism, GFR, renal tubular function, and histopathology before and 18-36 mo after OLT in eight patients with HTI. Progressive renal dysfunction was not documented despite continuing, although diminished, urinary succinylacetone excretion in all patients. The mean GFR was 82 mL/min/1.73 m 2 before and 102 mL at 18 mo and 93 mL at 36 mo after OLT. All patients showed tubular dysfunction before OLT. At 18 mo, glucosuria occurred in one, amino aciduria and phosphaturia in three, and hypercalciuria in six patients. Only hypercalciuria was seen at 36 mo. Renal biopsies showed mild Type I tyrosinemia is an autosomally recessively inh erited disease caused by FAR deficiency (1). FAR catalyzes the last step of tyrosine metabolism, and the deficiency leads to the accumulation of maleyl-and fumaryl acetoacetate and to the appearance of SA and SAA. Increased urinary excretion of SA and SAA occurs only in tyrosinemia and is diagnostic for the disease. The gene coding for FAR has been assigned to chromosome 15q23-q25 (2). Seven separate disease-inducing mutations of the gene have been described (3-6).Clinically type I tyrosinemia can be divided into two forms. The acute form of the disease is ch aracterized by signs of severe liver failure-ascites, bleeding tendency, and hypoglycemia-from the first months of life , and previously the patients died in early infancy. The chronic form is milder, and liver function less abnormal. However, the risk of hepatocellular carcinoma is increased, and carcinoma has been estimated