The first description of phlegmasia caerulea dolens of the leg due to deep vein compression by a giant mesenteric cyst is presented. In particular, the authors stress the role of the drainage of the cystic mass, performed as an emergency procedure, in the rapid improvement of symptoms, prevention of complications and achievement of definitive recovery. On the basis of such unusual case, a wide review of the literature on this subject is reported, aiming to stress the most crucial aspects regarding aetiology, clinical features, complications, therapy and prognosis of this disease.