Polycystic liver disease is a hereditary liver disorder which may present independently or as a consequent symptom of autosomal dominant polysystic kidney disease or autosomal recessive polycystic kidney disease. The pathophysiology remains complicated and yet to be understood completely which makes it difficult to standardize diagnostic and management protocols. Gigot classification and Schnelldorfer classification are used to determine severity in PLD. Clinical presentation of PLD in most patients remains ill defined and only a few patients present with complications and need the treatments. However various imaging studies such as magnetic resonance imaging and computed tomography makes it possible to identify and diagnose PLD at ease. The management strategies include somatostatin analogues, ursodeoxycholeic acid and vasopressin 2 receptor antagonists are potentially effective while fenestration, cyst aspiration and sclerosis, hepatic resection and liver transplantation are other invasive therapeutic options. This review focuses on briefing the classification, diagnostic techniques, and various management strategies to obtain potential directions for future researches.