Dialysis disequilibrium syndrome (DDS) is a neurological disorder with varying severity that is postulated to be associated with cerebral oedema. We described a case of DDS resulting in irreversible brain injury and death following acute haemodialysis. A 13-year-old male with no past medical history and weighing 30kg, presented to hospital with severe urosepsis complicated by acute kidney injury (Creatinine 1422mmol/L; Urea 74.2mmol/L, Potassium 6.3mmol/L, Sodium 137mmol/L) and severe metabolic acidosis (pH 6.99, HC03 1.7mmol/L). Chest radiograph was normal. Elective intubation was done for respiratory distress. Acute haemodialysis performed due to refractory metabolic acidosis. Following haemodialysis, he became hypotensive which required inotropes. His Riker's score was low with absence of brainstem reflexes after withholding sedation. CT Brain showed generalised cerebral oedema consistent with global hypoxic changes involving the brainstem. The symptoms of DDS are caused by water movement into the brain causing cerebral oedema. Two theories have been proposed: reverse osmotic shift induced by urea removal and a fall in cerebral intracellular pH. Prevention is the key to the management of DDS. It is important to identify high risk patients and haemodialysis with reduced dialysis efficacy and gradual urea reduction is recommended. Patients who are vulnerable to DDS should be monitored closely. Low efficiency haemodialysis is recommended. Acute peritoneal dialysis might be an alternative option, but further studies are needed.