Epidural varicosis is a rare though well-known cause of cauda equina syndrome (CES). Although inferior vena cava (IVC) obstruction is the most common finding in such cases, portal vein hypertension can lead to epidural venous plexus engorgement by means of lumbar portocaval shunt activation.A 40-year-old woman presented with right-sided sciatica, which progressed to right foot drop and a 3-day history of vesical tenesmus and fecal retention. She was initially diagnosed with L4-5 lumbar disc protrusion. However, contrast-enhanced lumbar MRI scan showed the presence of epidural varices in the L3-S1 tract. Given the absence of vascular anomalies amenable to resection, etiological conservative treatment was addressed. Therefore, a complete diagnostic workup was performed and revealed deep vein thrombosis (DVT), pulmonary embolism, and portal vein thrombosis. Oral anticoagulant therapy was initiated and prompt resolution of CES was observed. To the authors’ knowledge, this is the first report of CES secondary to epidural varicosis in the setting of acute portal vein thrombosis and extrahepatic portal vein obstruction (EHPVO). In cases of epidural varicosis, conservative etiological treatment is the most appropriate choice as CES may be the epiphenomenon of underlying systemic pathophysiological processes.