Font Size: 

Abstract. Polycystic liver disease (PCLD) is a rare condition in which multiple cysts form in thehepatic parenchyma. Polycystic liver disease is also an autosomal dominant disorder (ADPLD) caused by a mutation in a gene that encodes a protein hepatocystin. Given twogenes that are mutated in PCLD PRKCSH genes and gene SEC63. Mutations of these genes can be found less than a third of cases. The disease is rare. PCLD has a prevalence rate of 1: 200,000 people in the American population. PCLD occurs ± 24% of patients in the third decade of age to 80% by the sixth decade. Women tend to suffer larger cysts and more and correlated with the number of pregnancies. PCLD diagnosis is confirmed by ultrasound, CT scan, or MRI. Polycystic liver disease occurred in 95% of asymptomatic patients. The remaining 5% cause symptoms due to local effects of mass suppression polycystic liver or stretching or compression of the structure of other organs. PCLD treatment is not necessary if the patient is asymptomatic. Cysts were localized resectable, while diffuse to do the transplant. The following case report of a woman, 51 years old who were treated at the department of H.Adam Malik Medan on August 30, 2016 with a diagnosis of polycystic liver disease with right pleural effusion. Some literature has reported complications of polycystic liver disease, but rarely reported with pleural effusion presentation. Patients already done puncture of pleural fluid and after 3 weeks of treatment patient’s condition improved and permitted to be outgoing patient with her own request but never control back again.Key words: Policystic Liver Disease, Pleural Effusion