Three cases of cystic adventitial disease (CDA) of the popliteal artery and the results of a literature review are described in order to identify the treatment of first choice of this rare clinical entity. Three male patients (mean age 56,3 years) presented at our Institution due to a sudden abrupt of disabling claudication of the lower limb. The eco-color-Doppler examination showed multiple hypoechoic and anechoic masses arising in the wall of the artery consistent with CAD. The magnetic resonance imaging (MRI) confirmed this finding. In 2 cases a venous interposition grafting was performed and in the remaining patient a duplex-directed punction. The 3 patients are asymptomatic at the clinical control at 9 months after treatment. As it results after a literature review, the MRI seems to be the best diagnostic tool and the vein interposition grafting the treatment of first choice. In cases of young male patients without cardiovascular risk factors and suffering of disabling claudication of recent onset the MRI guarantees a valuable diagnosis in case of ultrasonographic suspect of CAD of the popliteal artery. The surgical resection and the vein interposition grafting represent the treatment of first choice. In selected patients, a duplex-guided punction assures satisfactory clinical results. A strict ultrasonographic follow-up guarantees an early recognition of relapse.