Abstract
Acute urea cycle disorder (UCD) presentation in patients can be challenging to manage, and the outcome can be deleterious if not promptly detected and urgently managed. This disorder is rare and can either be inherited or acquired. More descriptions of this disorder are noted in the paediatric population. We present a possible clinical scenario of acquired UCD in a middle-aged female with alcohol-related fatty liver disease who had a prolonged critical care admission, which culminated in an acute finding of a severe refractory acute hyperammonemia on her 67th day of critical care stay with a fatal outcome.