Background: The use of high-dose steroid therapy peri portoenterostomy may have a positive impact on the frequency of cholangitis and survival rate. Methods: A prospective study was conducted on two groups of patients (less than three months of age) suffering from biliary atresia from 1999 to 2005. The patients in group I (G I) were managed peri-operatively by high-dose methylprednisolone while the other group (G II) received low dose methylprednisolone only post-operatively (2mg/k/day for 1 month). Infants in GI (n=30) received methylprednisolone for 3 successive days before operation (10-8-6mg/kg/day), and 10 mg/k at the day of operation respectively. Thereafter the dose was tapered in the next successive 6 days by 8, 6, 5, 4, 3, and 2 mg/kg/day and continued for one month. Results: Seventy two infants with biliary atresia were operated (39 girls and 33 boys). Twenty-six of the 30 patients (86%) in G I became jaundice-free within 90 days after portoenterostomy while only seven (15%) of the 42 patients in G II had normal bilirubin (p <0.0001). Episodes of postoperative cholangitis in G I were 20% (6 of 30), and 53% (24 of 42) in G II (p <0.005). The difference in 3-year survival rate between the two groups is also remarkable: Eighty seven percent (26 of 30) in GI versus 29% (13 of 45) in G II (p <0.005). Death related to biliary atresia occurred in 1 (3.3%) patient in GI compared with 12 (29%) patients in G II (p <0.005). Conclusion: These results provide strong evidence that peri-operative high dose steroid therapy is not only safe in this patients population, but because of its anti-inflammatory and cholerrhetic effects has a positive impact on preventing recurrent cholangitis, and ultimately survival.