Granulomatous amoebic encephalitis caused by Balamuthia mandrillaris i
s rare (63 human cases reported worldwide) and fatal. We report a case
in a five-year-old boy who had previously been well. For 18 months, h
e had had a slowly progressive, granulomatous mid facial lesion, but d
espite extensive investigation definitive diagnosis was made only with
the acute onset of neurological signs in the last two weeks of life,
when a brain biopsy specimen revealed amoebic trophozoites and cysts.
infection with B. mandrillaris should be considered in the differentia
l diagnosis of chronic skin lesions with non-specific granulomatous hi
stopathology and negative microbiological test results.