Jaundice in older children can occur when any obstruction is found within the bile ducts, either from bile stones, parasites, choledochal cysts and rarely, secondary to tumors. We present a previously well, 10-year-old Filipino boy with three-week history of progressive jaundice and tea-colored urine, and was initially assessed to have biliary ascariasis. Ultrasound showed a heterogeneous focus spanning the gallbladder neck and confirmed on endoscopic retrograde cholangiopancreatography as an exophytic mass at the ampulla of Vater. Tumor biopsy and immunohistochemical staining confirmed the diagnosis of Embryonal Rhabdomyosarcoma of the Bile Ducts. Chemotherapy was initiated to reduce the size of the tumor before any surgical intervention could be attempted. Despite chemotherapy, the tumor progressively grew. He acquired a respiratory infection which led to sepsis and his eventual demise. The disease should be considered in the differential diagnosis of a child with obstructive jaundice after exclusion of other more common causes.