Compression of the left common iliac vein by the overlying right common iliac artery is a benign anatomic abnormality in most individuals. However, in patients with significant vein compression, outflow obstruction and chronic intraluminal venous damage may lead to May-Thurner Syndrome. This syndrome commonly manifests as unilateral left leg swelling or acute iliofemoral deep venous thrombosis. In addition to clinical findings, diagnosis is made with ultrasound, computed tomography venography, or magnetic resonance venography. The extent of compression of the iliac vein is best determined by venography with intravascular ultrasound. Symptoms and hemodynamic significance of the compression guides the ideal treatment approach. Iliocaval stenting has become the standard treatment for this condition and has promising patency rates and clinical outcomes. This review paper provided an overview of pathophysiology, and utility and limitations of the existing diagnostic modalities and treatment options in the management of May-Thurner Syndrome.