BACKGROUND Hypertensive encephalopathy is an uncommon neurological syndrome in children, usually with reversible clinical and neuroimaging findings. Little is known about the precipitating factors, clinical presentations, neuroimaging findings and outcomes of childhood hypertensive encephalopathy in Taiwan. METHODS To characterize this syndrome, we retrospectively analyzed 12 children with hypertensive encephalopathy in a tertiary institution from 1998 through 2005. We investigated the precipitating factors, clinical findings, courses, neuroimaging characteristics and outcomes. RESULTS Twelve patients (10 boys and 2 girls) with hypertensive encephalopathy were identified. Post-streptococcal glomerulonephritis was the most common precipitating underlying disease. Common clinical presentations included mental change (100%), seizure (91.6%), headache (66.6%), nausea or vomiting (75%), and blurred vision (41.6%). Brain imaging studies showed vasogenic edema over the bilateral parietal, occipital and parasagittal regions, or the cerebellum. All patients had a reversible clinical course. CONCLUSIONS Hypertensive encephalopathy is predominant in males, and mental change is the most common clinical presentation. Renal origin is a common precipitating factor. A characteristic lesion of hypertensive encephalopathy is occipitoparietal region edema. The overall clinical outcome is good after prompt treatment.