INTRODUCTION: Cortical laminar necrosis (CLN) is a specific pattern of neuronal death due to sparsity of glucose and oxygen. These lesions can cause persistent neurological deficits, even after treatment of the triggering etiology. Among various causes of CLN, data on hepatic encephalopathy manifesting as CLN is scarce. Here we report a case of acute hepatic encephalopathy presenting as CLN. CASE DESCRIPTION/METHODS: 63 years old lady with history of stroke, alcohol abuse and nonalcoholic steatohepatitis leading to cirrhosis, was brought in after being found unresponsive at home. She had a Glasgow Coma Score of 7 and was emergently intubated. Initial lab and imaging investigations led to a diagnosis of hepatic encephalopathy. Day 1 MRI (Figure 1) showed restricted diffusion in both cerebral hemispheres and multiple vascular territories suggesting hepatic encephalopathy. Patient’s condition kept deteriorating despite aggressive treatment with lactulose, rifaximin initially, plus renal replacement therapy from day 2. MRI done on day 6 (Figure 2) showed new patchy bright signal on FLAIR and T2 images and white matter of both cerebral hemispheres as well as the left pons. Asymmetry of hippocampal formations was seen and thought to be secondary to swelling and enlargement of left hippocampal guidance and suspected atrophy of right hippocampal formation. There was also a mass effect causing a 5 mm midline shift to the right which was related to suspected brain swelling on the left. These findings were consistent with cortical laminar necrosis. Palliative care team was taken on board and eventually the family decided to pursue a comfort pathway and patient was compassionately extubated. She passed away 8 days after admission on the day of extubation. DISCUSSION: One of the possible reasons for the mortality in our case could be that we were unable to sufficiently lower ammonia levels early in the course of acute illness until renal replacement therapy was started. There is scarcity of clear evidence in literature on whether early and aggressive medical therapy leads to better outcomes in these patients. Moreover, further studies are needed to define the “early” intervention and what would be the target level of ammonia before resorting to advanced therapies including renal replacement therapy in management of this disease.Figure 1.: Areas of restricted diffusion involving bilateral (left worse than right) temporal, frontal and parietal lobes. Old right temporal-parietal infarct.Figure 2.: Extension of restricted diffusion and development of extensive FLAIR changes in bilateral temporal, parietal and frontal lobes. Old right temporal-parietal infarct.