Objective: To analyze the clinical data of a patient diagnosed as autosomal dominant polycystic kidney disease (ADPKD) complicated with pulmonary embolism (PE), and review reported cases in data base to explore the diagnosis and treatment in the ADPKD patients with venous thromboembolism(VTE). Methods: A 45-year-old male diagnosed as ADPKD [also had polycystic liver disease (PLD)] and pulmonary embolism (PE) was admitted in our hospital, and the clinical manifestations and laboratory results were analyzed. The diagnosis and treatment in the patients with ADPKD complicated with VTE were reviewed through searching PubMed and entire database. Results: The ADPKD patient with PLD, presenting with chest pain and shortness of breath, was diagnosed as PE using pulmonary ventilation perfusion examination and as deep VTE through imaging of deep venous. The MRI enhancement of the upper abdominal organs showed the stenosis lesions in hepatic segment of inferior vena cava, and polycystic liver combined with polycystic kidney. The heterozygous variation of PKD1 gene and PROS1 gene were detected by the whole exon sequencing. A total of 15 ADPKD patients complicated with deep VTE were reviewed. Among them, all cases had inferior vena cava thrombosis (IVCT), 13 cases had PLD, 2 cases had hepatic vein thrombosis, 12 cases had VTE, and 2 cases had PE. The symptoms of 10 ADPKD patients were relieved after anticoagulation treatment with heparin and warfarin, while 5 patients needed hardener or inferior vena cava filter placement or surgical treatment. Conclusions: The polycystic kidney(PKD) and PLD are not considered as the most common causes for VTE, which are easy to be ignored in medical practice. The ADPKD patients usually complicated with PLD, which may cause compression of inferior vena cava and lead to embolism. The primary treatment is anticoagulation, and the adjuvant therapy is relief of cyst compression.