Aim and background: Urea cycle disorder (UCD) is a metabolic disturbance and can be a harbinger of life-threatening hyperammonemic encephalopathy. We report a case of adult-onset partial urea cycle defect precipitated by septic shock and status epilepticus and its successful treatment with continuous renal replacement therapy, adaptive nutrition, nitrogen scavengers, and gut decontamination. Case description: A 37-year-old woman with known seizure disorder presented with status epilepticus, urosepsis, and septic shock due to Enterococcus faecalis infection. Her two other sisters died from an unknown neuropsychiatric illness in early childhood. She was initially treated with invasive mechanical ventilation, vasopressor support, intravenous antibiotics, and antiepileptic drugs. After stabilization of hemodynamics and control of visible seizures, her mental status was not improving despite the sedation hold. Further workup revealed high ammonia, respiratory alkalosis, triphasic waveform on the electroencephalogram, normal liver function and sonologic evidence of cerebral edema. Urinary analysis for amino acids and organic acids was noncontributory. A partial defect in the urea cycle was suspected and she was successfully treated with continuous renal replacement therapy and other available adjunctive treatments to a favorable outcome. Conclusion: In patients who present with noncirrhotic hyperammonemic encephalopathy and have had one or multiple stressors, a diagnosis of UCD should be suspected to optimize early management and improve prognosis.