We report on a case of severe cystic adventitial disease involving the popliteal artery in a man aged 42, who in 2009 underwent surgical resection of the cyst and the tract of the involved artery. The arterial reconstruction was then performed with an autogenous graft, using a reversed small saphenous vein interposition. The surgical specimen was histologically examined. The postoperative course was uneventful, and the patient was discharged on the 8th day. Currently, on an 11-year long clinical follow-up, the patient is healthy, and the graft is patent. A pictorial essay of the clinical and pathological findings of the case is presented, along with a brief discussion covering the main issues relevant to the disease manifestations and diagnosis, clinical differential diagnoses, and the treatment options. The pertinent world literature has been reviewed as well.