Objectives: This study was undertaken to know about the magnitude, clinical spectrum and etiology of liver diseases in children in a tertiary care teaching hospital. Materials and Methods: This is a hospital based, retrospective, descriptive study. It was done on 45 children with hepatobiliary diseases admitted to pediatric department of Velammal Medical College, Madurai from Jan 2016-Dec 2016. Results: As about 45 children out of 2259 pediatric admissions, had hepatobiliary disorders. This was contributing to 1.99% of total admissions. Liver function test was deranged in (73.3%) of children. Males (62.2%), outnumbered, females (37.7%) in our study. Children less than 5 years of age were most commonly affected (46.6%). Most common symptom in children with hepatobiliary disorders were jaundice (51.1%), nausea/vomiting (46.6%), anorexia (40%), pain abdomen (33.3%), high coloured urine (28.8%), bleeding manifestations and abdomen distension in (20%) each, fever in (17.7%), failure to thrive and irritability in (15.5%) each. The most common sign observed was icterus (51.1%), hepatomegaly (42.2%). Ascitis and splenomegaly was noted in (26.6%) each, edema in (20%) and pallor in (15.5%) of children. Acute liver diseases were more common (68.8%) than chronic liver diseases (31.1%). The most common etiology of acute liver disease was acute viral hepatitis (28.8%), cholelithiasis (22.2%), non-alcoholic fatty liver disease (6.6%), liver abscess (4.4%), acute viral hepatitis with acalculous cholecystitis in (4.4%), acute liver failure due to paracetamol poisoning in (2.2%) of children. Etiology noted for chronic liver diseases were biliary atresia (13.3%), idiopathic cirrhosis and wilsons disease in (6.6%) each, autoimmune hepatitis and chronic hepatitis in (2.2%) each. Conclusion: Since age is the single most important determinant in successful management of biliary atresia, recognition and definitive identification of the condition as the cause of neonatal cholestasis syndrome in a given case very early after the onset of symptoms is of paramount importance.