Polycystic liver disease (PLD) is a rare inherited disorder that can exist independently, or as an accompanying symptom of autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease. The pathogenic genes and associated mechanisms of PLD are complex and diverse, which are still not completely clear, and are now considered to be associated with the developmental abnormalities of the bile ducts caused by various factors. PLD is characterized by multiple scattered liver cysts, which cause no symptoms in majority of cases, but will cause symptoms in some cases such as abdominal distention, tachypnea, esophageal reflux, abdominal pain and back pain as cysts grow in size and number, and thereby affect the quality of life. The use of ultrasound, CT or magnetic MRI can help to diagnose PLD. Among patients with no family history, the diagnosis of PLD can be made when the number of hepatic cysts exceeds 20. Furthermore, if genetic analysis is available, PLD can be diagnosed at the genetic level and classified. For symptomatic PLD patients, different treatment schemes can be selected according to the different general conditions and the ranges affected by the lesions. The treatments of PLD mainly include the pharmacotherapy and surgical intervention. For pharmacotherapy, studies have shown that somatostatin analogues, mammalian target of rapamycin, ursodeoxycholic acid, vasopressin 2 receptor antagonists have potentially therapeutic capacity, however currently only the somatostatin analogues are widely accepted and used in clinical practice, while the others need further researches to validate the safety and effectiveness. The commonly used surgical intervention includes cyst aspiration and sclerosis, fenestration, transcatheter arterial embolization, hepatic resection, and liver transplantation, in which liver transplantation is the only method of radical treatment. However, it should be noted that there is no breakthrough in diagnosis and treatment of PLD at percent. In this article, the authors address the progress of clinical diagnosis and treatment of PLD and comprehensive information of clinical application of the treatments in order to deepen the understanding of the current clinical diagnosis and treatment of PLD and guide the future direction. In addition, the diagnosis and treatment process according to the experience of our medical center are provided for reference.