Abstract Background Delirium is an acute, usually reversible neuropsychiatric syndrome and often presents as confusion, especially in the older population. Early detection and management of causes is vital to reduce mortality, morbidity, and long-term cognitive status decline. Intrahepatic portosystemic venous shunts are rare vascular malformations. These malformations can be congenital or acquired and may manifest as heart failure, hepatic encephalopathy, or pulmonary hypertension. Methods A 75-year-old man presented to the emergency department with a 5-week history of confusion, daytime somnolence, and fatigue. His physical examination was unremarkable. His symptoms were initially thought to be due to sub-optimally controlled Obstructive Sleep Apnoea. Nevertheless, his confusion progressed further during admission and required admission to the intensive care unit and intubation. Investigations initially showed deranged Liver function tests. Extensive investigations were done, including CT brain, MRI brain, septic screen, Arterial blood gas, and Lumbar puncture, which were all inconclusive. An Electroencephalogram was suggestive of Encephalopathy. His Ammonia level was high (123). Further investigations, Triphasic CT liver, revealed Intrahepatic portosystemic venous shunt and was diagnosed with hyperammonaemic hepatic encephalopathy due to Intrahepatic portosystemic venous shunt. Results Management included the commencement of lactulose, rifaximin, and L-ornithine L-aspartate (LOLA). An IR-guided embolisation of the porto-venous shunt was performed. At two-months review, the patient reported no further episodes of confusion. Conclusion Intrahepatic portosystemic venous shunts should be considered in the differential diagnosis of patients presenting with confusion or other unexplained neurological symptoms, especially in the presence of liver dysfunction. Early diagnosis and appropriate management are crucial for favourable outcomes.