Background: Lymphoblastic lymphoma (LBL) presenting with obstructive jaundice cases are rare. It is rarely considered in the differential diagnosis of patients with obstructive jaundice. Case description: A young woman complained of jaundice, tea-colored urine, pale stools, and lost weight. The abdominal examination showed mild tenderness in the epigastric area. Laboratory showed elevation of liver function, bilirubin, and alkaline phosphatase. The radiological examination both in abdominal ultrasound and magnetic resonance cholangio-pancreatography suggested a possible differential diagnosis between of lymphoma and cholangiocarcinoma. She underwent surgery for open biopsy from biliary duct and showed lymphoblastic lymphoma. She was received combination of chemotherapy. She had significant improvement of her quality of life. Conclusion: The diagnosis of biliary duct lymphoblastic lymphoma is essential but can be very challenging. Clinical diagnosis is based on symptoms and signs, while laboratory testing is non-specific and occasionally misleading. However, direct imaging and biopsy guidance is highly recommended.