Surgical treatment of neonatal cholestasis is best illustrated in two particular pathologic conditions: biliary atresia and congenital choledochal cyst. Almost a century passed since the first attempts of treating children’s cholestasis were undergone. The ethiology and pathology of these conditions were also poorly understood. The first surgical intervention specifically designed for the correction of biliary atresia was described by Morio Kasai in 1959. Actually, the surgical treatment of both biliary atresia and congenital choledochal dilatation are well established: Kasai’s portoenterostomy and, accordingly, cyst excision and Roux-en-Y hepaticojejunostomy. Unfortunately, the overall poor results after portoenterostomy make liver transplantation an ultimate and mandatory solution for the long-term survival of the patients.