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.