May-Thurner syndrome presenting as spontaneous left iliac vein rupture

Spontaneous iliac vein rupture resulting in a retroperitoneal hematoma is extremely rare and can present as a life-threatening emergency. There is often a delay in diagnosis with no established treatment recommendations. We report the case of a 39-year-old woman who presented with hypovolemic shock, a large left retroperitoneal hematoma, and left lower extremity phlegmasia in the setting of a previously asymptomatic May-Thurner syndrome. She was successfully treated with a combined open and endovascular approach. We also reviewed the literature on the evolution of diagnosis and treatment of this rare condition and present our recommendations for management.