Uremic encephalopathy is an absolute indication to start renal replacement therapy. Hence, early diagnosis of UE is essential. Uremic encephalopathy is a clinical syndrome with a variable and subtle presentation. There is no confirmatory test, which leads to a delay in diagnosis. During the evaluation, it is essential to exclude conditions that may mimic UE, such as infection, osmotic demyelination, subdural hematomas, hypertensive encephalopathy, cerebrovascular accidents, disequilibrium syndrome, amongst others.