We report a 12 years old female patient with an overlap syndrome involving 
autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient 
presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis 
and butterfly rash on the face. Laboratory tests revealed severe liver 
dysfunction, Coombs positive hemolytic anemia and a positive ANA/anti-dsDNA 
test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed 
chronic hepatitis with severe inflammatory activity. The patient satisfied the 
international criteria for both SLE and AIH. Clinical symptoms and laboratory 
findings of SLE improved with high dose treatment with corticosteroids and 
azathioprine, however, remission of the liver disease could not be achieved. 
Repeat biopsy of the liver after three years of therapy revealed ongoing chronic 
hepatitis with high level of inflammatory activity. The present case indicates 
that children with liver dysfunction and SLE should be investigated for AIH. 
There is much diagnostic and therapeutic dilemma in patients with AIH-SLE 
overlap syndrome.