The leading cause of pediatric liver failure and liver transplantation is biliary atresia. Early diagnosis of BA is challenging, and delayed diagnosis caused many complications. Most BA patients eventually went through liver transplantation even if received the initial treatment of the disease. The incidence of BA is rare hence limited literature has been published about diagnosis, treatment, risk factors and survival rate among BA patients. Therefore, this literature review aimed to provide an opportunity for physicians and researchers to know about different diagnostic and treatment tools as well as about the risk factors causing variation in the survival rate after liver transplantation. Therefore, knowledge about the advanced methods of diagnosis provides an early and accurate diagnosis and providing treatment with fewer risk factors help to increase favorable outcome and increase the survival rate among BA patients.