<p> Wilson disease is a rare, inherited autosomal recessive disease of copper metabolism. It may be more common where consanguinity is prevalent. It occurs due to dysfunction of a copper transporting p-type ATPase that has a crucial role in copper excretion in bile. The gene that encodes this ATPase, is located on chromosome 13q14.3. This leads to copper accumulation in liver, brain, cornea and kidney. The most frequent clinical presentation of WD is liver involvement. Wilson disease in children poses diagnostic challenges, as many other conditions can present similarly. Even at biopsy, definitive diagnosis may be difficult. We attempted to study the role of liver biopsy in Wilson disease, assess close differential diagnosis, and take note of the limitations of liver biopsy in diagnosing liver biopsy. Nine out of ninety five liver biopsies done over a 5 year period, and diagnosed as Wilsons disease were selected, based on the clinical presentation, investigative findings and biopsy features. Liver biopsy helped in identifying cirrhosis, assessing compliance and response to treatment. Close differential diagnoses were viral hepatitis and auto immune liver disease. While ballooning of hepatocytes and nuclear glycogenation were consistently found, not all cases showed classical features of Wilson disease, making biochemical investigations as important as the clinical opinion and biopsy findings, all three forming a diagnostic triad.</p>