It has been suggested that cortisol deficiency may be responsible for the cholestasis. Although congenital hypopituitarism was reported as a possible cause of neonatal cholestasis, congenital adrenal hyperplasia with neonatal cholestasis is a very rare report in the literature. We experienced a case presenting with neonatal cholestasis associated with congenital adrenal hyperplasia. This case provides a clinical evidence supporting the notion that cortisol has an important physiological role in bile acid synthesis and transport.