The popliteal artery entrapment syndrome is characterized by extrinsic compression of this artery as a result of anatomic deviation from its usual course, or by compression from musculotendinous structures in the popliteal fossa. Clinical symptoms appear when these affected individuals do strenuous exercises. There are two types of popliteal artery entrapment syndrome: the classicalor congenital form, and the functional or acquired form. In the classical form, disturbances in the embryogenesis lead either directy to popliteal artery anomalies or to alterations of adjacent structures that cause compression of the popliteal artery. In the functional form, hypertrophy of the gastrocnemius muscle secondary to exercise has been postulated as a cause. In both types, diagnosis is made through the detection of total occlusion or important stenosis of the popliteal artery. This is identified by duplex scan, magnetic resonance or arteriography during active plantar flexion-extension. A positive test in non-symptomatic subjects presenting no anatomical anomalies led to discussions about its specificity. This study presents a review of anatomical and functional popliteal artery entrapment syndrome and discusses accuracy, sensitivity and specificity of the diagnostic tests.