Exercise-induced arterial endofibrosis (EIAE) is now a 20-year-old concept. Initially observed in highly trained cyclists, it has been found in many other male and female endurance athletes. Most stenoses are located on the first centimetres of the external iliac artery but other localisation may be found. The disease is defined by specific histological findings showing fibrosis of the intimal and medial wall of the artery. Ankle pressure measurement plays a key role in the diagnosis of EIAE provided that the exercise tests reproduce symptoms and are performed with the subject being highly trained, and that pressure measurements are performed early following exercise. Various cut-off values have been proposed in the literature.Magnetic resonance imaging or arteriography or ultrasound imaging should be performed by trained operators only. Surgical ‘endofibrosectomy’ is preferable in young cyclists generally associated with a saphenous enlargement patch. We do not recommend angioplasty. Athletes are generally allowed to return to competition within 3–4 months following surgery. Many unanswered questions remain, specifically about the pathophysiology of this disease. Mechanical and pressure strain play a central role in the development of EIEA, but nutritional and haemorrheological factors, hormonal changes and a genetic predisposition are likely to contribute. New techniques could facilitate the diagnosis of EIAE.