Kasai's portoenterostomy is, so far, the only option for patients with biliary atresia (BA) to survive with their own liver. The long-term results are closely related to the timing of the procedure and to the experience of the center. However, optimal conditions cannot guarantee stable liver function. Unfortunately, the majority of patients with BA eventually need liver transplantation, making them the largest group of pediatric organ recipients. Thus, surgery in patients with BA treats only the symptoms, but never the cause of the disease. In order to focus on this point, international and interdisciplinary cooperation is mandatory to improve early and effective diagnosis, to optimize surgical therapy, and to coordinate clinical and basic research in BA. Uncovering its unknown aetiology is crucial for developing and modifying new therapeutic attempts to treat the disease, including the opportunities for prophylaxis. Until then, surgical treatment is still the best approach for biliary atresia and, so far, no further prospects are apparent.