Three patients, two women aged 66 and 43 years, respectively, and a man aged 76 years, presented with sudden, painful, blue areas in the toes with intact peripheral pulsations. One patient had a myeloproliferative syndrome due to essential thrombocytosis, the second patient had thromboangiitis obliterans, and the third patient had a cholesterol embolism, possibly due to the use of oral anticoagulants. After treatment, one patient recovered fully and the other two improved significantly. The blue toe syndrome is the pathophysiological consequence of end-arterial occlusion and frequently the first manifestation of a systemic disorder, such as atheroembolism or vasculitis. Adequate treatment is possible in most cases. Therefore, it is very important to recognise this disorder and its possible causes so as to prevent further episodes of local symptoms and especially systemic complications.