ABSTRACT
Wilson disease is a hereditary disorder of copper metabolism which can present with hepatic, neurologic or psychiatric symptoms and in rare cases as a hemolytic disturbance. Free copper can result in red blood cell damage and hemolysis which is a rare feature of the disease affecting less than 10% of patients. In this condition the liver is usually involved and liver transplantation can be life saving. This article is a case report of acute hemolytic crisis as the initial manifestation of Wilson disease. An 8 years old girl was admitted in pediatric ward of Golestan hospital because of abdominal pain, icterus, anemia and tea color urine. Hepatitis or Glucose 6 Phosphate Dehydrogenase deficiency was the first diagnosis. Because of unresponsiveness to transfusion, Wilson disease was considered and the diagnosis was established with the presence of Kayser Fleischer ring. Chronic or acute hemolytic anemia is a rare or unusually presentation of Wilson disease. In any child especially older than 5 years with liver disease or hemolytic anemia, Wilson disease should be considered and appropriate diagnostic tests performed