Hydranencephaly with cholestasis and giant hepatitis.

We present a rare case of hydranencephaly with cholestasis and giant hepatitis. Studies for infectious agents were all negative including for the detection of virus in liver tissue by using polymerase chain reaction. Although the anterior pituitary functions (cortisol, thyroid stimulating hormone, free T4, human growth hormone) were normal, the patient revealed massive cholestasis and giant hepatitis.