A fatal case of Coxsackievirus B-6 (CBV-6) infection in a 4 1/2-year-old girl is reported. The disease was initially characterized by a severe meningoencephalitis and, successively, by the appearance of hyperglycaemia and glycosuria, concomitantly with complement-fixing-islet cell antibodies (CF-ICA) and ICA, diarrhoea, electrolyte disorders, arrhythmia and decrease of the IgG levels, suggesting a multi-system involvement. CBV-6 was identified by isolation from stool and cerebrospinal fluid and by detection of specific IgM antibodies.