Biliary atresia (BA) is a rare disease characterised by biliary obstruction of unknown origin that presents in the neonatal period. It is classified into syndromic with various congenital anomalies and non-syndromic (isolated anomaly). We present a case of syndromic BA associated with polysplenia and intestinal malrotation, discovered incidentally during the Kasai procedure. The small intestine was found to be non-rotated with the duodenojejunal junction to the right of the vertebral column. The presence of accessory spleens was noted. Kasai portoenterostomy and Ladd's procedure were performed. The patient had an uneventful postoperative course with the passage of cholic stool from the third postoperative day. At the seventh-month follow-up, the stool remained cholic. A multidisciplinary approach in the care of babies with BA and long-term follow-up is crucial for a successful outcome.