Introduction: Hepatobiliary manifestations are common in inflammatory bowel disease (IBD); frequently manifesting with abnormal hepatic biochemical tests. Of these disorders, primary sclerosing cholangitis (PSC) carries the most significant clinical implications and remains a highly challenging disease to manage. Hepatotoxicity can occur with nearly all medications in the treatment of IBD. Discontinuation of drug, or dose reduction in some cases, is indicated when abnormalities are detected. Aim of work: To determine the frequency of hepatobiliary manifestations in Egyptian patients with IBD. Patients and methods: The work was carried out at the pediatric hepatology, gastroenterology units, Cairo University Children’s Hospital, from April 2013 to September 2013. It included patients younger than 18 years of age, of both sexes. The available data was retreived from the patients’ files diagnosed as IBD as well as newly diagnosed patients. Data collected included: history taking, general, abdominal examination and anthropometric measures and investigations included CBC, liver functions, autoantibodies, serum immunoglobulins, abdominal ultrasound upper and lower endoscopies with histopathological examination and MRCP and liver biopsy as available. Results: The study included 48 patients; 69.5% of them had ulcerative colitis (UC) while 30.6 % had Chron’s disease (CD). Males represented 60.4% of the patients. Mean age of the patients was 10.16 ± 4.19 years. There was positive family history in 18.8% of the patients. Recurrent abdominal pain was the main presenting symptom being present in 97.9% of the patients, followed by diarrhea and bleeding per rectum. Pancolitis was present in 8 (17.4%) of the patients. P-ANCA was positive in 43.8/5, 37.6% of patients with CD and UC respectively. Elevated liver enzymes were observed in 16.3% of the patients, 20.8% of the patients had bright liver on abdominal ultrasound, while two patients had sclerosing cholangitis. Conclusion: Hepatobiliary manifestations of IBD are not uncommon and can be evaluated by liver function tests and abdominal ultrasound. PSC represents one of the most common and serious complications. Routine screening for these complications is mandatory as some of them could be asymptomatic.