Nickan Research Institute Journal of Parathyroid Disease 2345-6558 6 2 2018 04 01 Superior vena cava syndrome complicating calcific uremic arteriolopathy in an ESRD male patient on maintenance hemodialysis following failed kidney transplantation 68 71 10.15171/jpd.2018.21 EN Macaulay Amechi Chukwukadibia Onuigbo Nneoma Agbasi Abdul Khan Vinay Nijhawan Zhibin Jiang Journal Article 10.15171/jpd.2018.21 We described the unusual presentation of right unilateral facial swelling in a 48-year old end-stage renal disease (ESRD) male patient on maintenance hemodialysis following a failed kidney transplant. This was subsequently confirmed to be secondary to the extrinsic compression of the superior vena cava (SVC) by a large lobulated amorphous extra-osseous right axillary mass lesion that extended into the upper right thoracic outlet. Superior vena cava venogram and balloon angioplasty led to symptomatic relief. The factors involved in the pathogenesis of calciphylaxis are discussed and the available therapeutic options for this rare albeit debilitating disease are reviewed.