Two infants who died unexpectedly and who were found at autopsy to hav
e idiopathic arterial calcification are presented. The first infant di
ed within hours of the sudden onset of shortness of breath. The second
infant died suddenly and unexpectedly in hospital where he was being
treated for presumed sepsis and cardiac failure. Neither infant had si
gnificant past or family histories. Autopsy examination in both infant
s demonstrated widespread fibrointimal proliferation of elastic and mu
scular arteries with characteristic calcification of the internal elas
tic laminae. Kidneys and parathyroid glands were normal. Death in case
1 was attributed to extensive myocardial ischemic damage with right c
oronary artery ostial stenosis due to idiopathic arterial calcificatio
n. Death in case 2 was attributed to saddle pulmonary thromboembolism
arising from a right atrial thrombus associated with cardiac failure s
econdary to idiopathic arterial calcification. These cases demonstrate
the variable presentations, causes of death, and autopsy findings tha
t may occur in the uncommon condition.