eng Science and Education Publishing American Journal of Medical Case Reports 2374-216X 2017-07-13 5 6 160 162 10.12691/ajmcr-5-6-7 AJMCR2017567 article Laith Alkukhun 1 Ahmed Abu-Haniyeh abuhana@ccf.org 1 Mohammed Al-natour 2 Muhammad Hassan 3 Adriano Tonelli 4 Internal Medicine Residency Program, Medicine Institute, Cleveland Clinic, Cleveland, OH, USA Interventional Radiology Department, Imaging Institute, Cleveland Clinic, Cleveland OH, USA Jinnah Sindh Medical University, Karachi, Pakistan Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, OH, USA CLOVES syndrome (Congenital Lipomatous Overgrowth, Vascular malformations, and Epidermal nevi) is a rare and recently discovered syndrome. It is associated with vascular malformations and abnormal fatty tissue and skeletal growth. In this case, we report the clinical manifestations overtime in a patient with CLOVES syndrome who presented a unique arteriovenous malformation that led to severe neurological impairment by compressing the spinal cord. We also discuss the visceral findings in our patient, such as double ureter and splenomegaly, and describe the finding of corneal hydrops in both eyes and Chiari I malformation on brain imaging. http://pubs.sciepub.com/ajmcr/5/6/7/ajmcr-5-6-7.pdf CLOVES syndrome arteriovenous malformation disseminated intravascular coagulation