A female patient, who was diagnosed with Leigh syndrome at 15 months o
f age, developed fulminating severe hypertension and died at 8 years o
f age. Hypertension has not been reported as an important clinical sym
ptom in Leigh syndrome. Laboratory findings indicated that it was not
associated with endocrinopathic diseases such as pheochromocytoma and
aldosteronism, or renal diseases. Brain MRI scan showed symmetrical le
sions in the basal ganglia and medulla oblongata including the nucleus
tractus solitarius. This nucleus is known to play an important role i
n maintaining blood pressure. Since the medulla oblongata is a vulnera
ble site, potential development of hypertension should be taken into c
onsideration when managing Leigh syndrome.