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Polycystic liver disease is a rare inherited disorder which affects 1 in 1,00,000 population. There are two forms of polycystic liver disease (PLD) i.e. isolated PLD and autosomal dominant PLD which is associated with renal cysts. PLD is usually asymptomatic and incidentally detected. Some may present with complaints of abdominal distension, bloating, early satiety, weight loss. Computed tomography (CT) or magnetic resonance imaging is the investigation of choice to diagnose polycystic liver disease. Below we report you a case of 55-year-old female who came with the complaints of pain abdomen since 8 days and was diagnosed as polycystic liver disease on CT, who received treatment by laparoscopic fenestration surgery of cysts and showed symptomatic improvement.

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