Introduction: Esophageal variceal ligation (EVL) and sclerotherapy are both considered standards of care in management of esophageal variceal hemorrhage. Minor complications are not uncommon but occasionally, severe and potentially life-threatening sequelae may develop. Case presentation: Patient is a 30-year-old female with history of variceal bleeding due to portal hypertension secondary to noncirrhotic focal nodular hyperplasia (FNH) and resultant portal vein thrombosis that began at age 13 until she underwent banding at age 20. She was also treated with a splenorenal shunt as well as a superior mesenteric vein to inferior vena cava shunt to help reduce portal venous pressure. Evaluation for anemia with esophagogastroduodenoscopy (EGD) revealed an extremely large esophageal varix in the distal esophagus with active bleeding, which initially improved with sclerotherapy. Ten days after, she vomited blood and fainted. Methods: Repeat EGD noted profuse variceal bleeding, requiring intubation for airway protection. Attempts to band the varices were unsuccessful, requiring repeat sclerotherapy. 27 mL of sodium tetradecyl was injected throughout the esophagus. Post-procedure chest X-ray revealed a large left-sided pneumothorax, requiring a chest tube, and esophageal perforation. Portal venous shunt was attempted; however, the anastomosis was thrombosed, so the cryopreserved vein was ligated. Post-operative esophagram showed no obvious leak. CT chest/abdomen/pelvis showed bilateral pleural effusions. Left pigtail catheter was placed and drained 150 mL and grew E. faecalis as well as Candida kefyr; patient was placed on zosyn and fluconazole. Results: Patient was transferred to medical ICU intubated on CPAP, hemodynamically stable. Patient recovered with all catheters removed following prolonged antimicrobial support. She was stable on discharge and placed on beta blocker therapy without further incident. Conclusion: This case is unusual for many reasons. Firstly, the etiology of portal hypertension secondary to FNH; secondly, the continuous complications of portal hypertension despite previous shunting; and thirdly, her development of a massive upper gastrointestinal bleed a week after successful EVL. Although sclerotherapy is still occasionally used after unsuccessful EVL, we must be cognizant of rare but potentially devastating complications. For this patient, prompt recognition followed by appropriate and aggressive management fortunately resulted in a successful outcome with long-term survival.