The coexistence of achalasia and gastroesophageal varices has been reported sporadically in the English medical literature. We report the case of a 60-year-old Hispanic woman with cryptogenic cirrhosis who was referred for a liver transplant evaluation and subsequently developed progressive dysphagia to both solids and liquids as well as substernal chest pain and weight loss. Endoscopy revealed the presence of grade I esophageal varices and large fundic varices, as well as retained liquid and solid food in the distal esophagus. Radiographic and manometric studies were consistent with achalasia. After botulinum toxin (Botox) injections were no longer effective a transjugular intrahepatic portosystemic shunt was performed for portal decompression before proceeding with pneumatic dilation. Optimal treatment of these 2 conditions, when they occur simultaneously, is problematic. We discuss this patient’s management and our approach to this infrequent combination of diseases. The coexistence of achalasia and gastroesophageal varices has been reported sporadically in the English medical literature. We report the case of a 60-year-old Hispanic woman with cryptogenic cirrhosis who was referred for a liver transplant evaluation and subsequently developed progressive dysphagia to both solids and liquids as well as substernal chest pain and weight loss. Endoscopy revealed the presence of grade I esophageal varices and large fundic varices, as well as retained liquid and solid food in the distal esophagus. Radiographic and manometric studies were consistent with achalasia. After botulinum toxin (Botox) injections were no longer effective a transjugular intrahepatic portosystemic shunt was performed for portal decompression before proceeding with pneumatic dilation. Optimal treatment of these 2 conditions, when they occur simultaneously, is problematic. We discuss this patient’s management and our approach to this infrequent combination of diseases.