This report describes a patient with neonatal lupus erythematosus (NLE) in whom there was multisystem involvement including neonatal hepatitis. The hepatitis, defined pathologically as a giant cell hepatitis, presented with severe cholestasis, was unrelated to other known causes of neonatal hepatitis and resolved spontaneously by 6 months of age. Both mother and infant were positive for Sjögren syndrome A+B antibodies (SS-A[Ro] and SS-B[La]) as well as having high titres of antinuclear antibody (ANA). Three of the four cases described in the one previous report of this association were also ANA positive. This antibody may be a marker for the development of the hepatitis. Maternal and infant ANA status should be determined in cases labelled as idiopathic neonatal hepatitis to exclude undiagnosed maternal SLE as a cause of neonatal cholestasis.