An infant with prolonged circumcision bleeding and unexplained coagulopathy.

Tyrosinemia type I is a rare autosomal recessive disorder. Fulminant onset of liver failure can occur in the first few months of life. Because all of the clotting factors are produced exclusively in the liver except factor VIII, coagulation abnormalities are very common in patients with severe liver disease. Rarely a significant coagulopathy in the absence of overt signs of liver disease may be seen in hereditary tyrosinemia. We present a 4 weeks-old tyrosinemic infant who presented with severe bleeding after circumcision and no other signs of liver failure. The diagnosis of tyrosinemia should be kept in mind in differential diagnosis of bleeding disorders especially a severe coagulopathy unresponsive to vitamin K, and fresh frozen plasma, even when other signs of liver failure are absent.