Aim: To describe hepatobiliary involvement in pediatric hematolymphoid malignancies and their approach in clinical practice. Background: Pediatric hematolymphoid malignancies primarily consist of leukemias, lymphomas, and Langerhans cell histiocytosis (LCH). Although they involve the hepatobiliary system frequently, they are often misdiagnosed as infectious or inflammatory diseases. Also, consolidated literature on their presentation and approach is scarce in children. Review results: Hepatobiliary involvement is seen in up to 60% of acute leukemias and LCH and up to 40% of lymphomas in children. Clinical features result from infiltration, compression, overwhelmed immune system, and chemotherapy-related hepatotoxicity. Hepatobiliary involvement in hematolymphoid malignancies is diverse, ranging from subtle asymptomatic hepatomegaly, raised transaminases, and biliary obstruction to fulminant presentations like liver failure and decompensated biliary cirrhosis due to sclerosing cholangitis. The majority of the chemotherapeutic drugs have the potential for hepatic impairment, necessitating pharmacovigilance. Conclusion: Hepatobiliary involvement in children can present with a wide range of manifestations, from asymptomatic hepatomegaly to fulminant liver failure. Pivotal for favorable outcomes is to arrive at an early diagnosis of malignancy by differentiating it from inflammatory and infectious diseases. Hepatotoxicity can be reduced or even prevented by practicing pharmacovigilance. Clinical significance: Liver involvement in hematolymphoid malignancies often overlaps with common infectious and inflammatory diseases requiring a high index of suspicion. It is essential to improve cross-referrals between a hemato-oncologist and a pediatric gastroenterologist for optimal outcomes.