The ketogenic diet has demonstrated good efficacy in children with pharmaco
logically resistant seizures. Relatively few serious complications have bee
n reported in the more than 70 years in which the diet has been used. We re
port a child who developed acute pancreatitis and died. A 9-year-old girl h
ad a seizure disorder with associated developmental delay owing to glucose
transport protein deficiency. The ketogenic diet with medium chain triglyce
ride oil had been initiated shortly after diagnosis in infancy. She was not
on anticonvulsants. She presented in coma with decreased respiratory effor
t and shock, requiring resuscitation. Investigations were consistent with p
ancreatitis. Despite fluid resuscitation and inotropic support, she had pro
longed hypotension and acidosis. She subsequently had a cardiac arrest and
died. A postmortem examination confirmed hemorrhagic pancreatitis. Hypertri
glyceridemia is a risk factor for developing acute pancreatitis. The high f
at content of the ketogenic diet often causes hyperlipidemia. The outcome f
or this patient raises concern regarding a potential consequence of the ket
ogenic diet.