Abstract
The urea cycle is a metabolic pathway that excretes nitrogenous waste products from the body. Urea cycle disorders (UCDs) result from enzymatic deficiencies within this pathway, which can lead to life-threatening hyperammonemia. Gastric bypass-related hyperammonemia in patients who have undergone Roux-en-Y gastric bypass surgery has been previously reported. UCDs have been implicated as a cause of gastric bypass-related hyperammonemia. In this report, we present the case of a patient with a history of bariatric surgery who experienced severe hyperammonemia encephalopathy triggered by a gastrointestinal bleed due to an undiagnosed UCD.