Lipoid proteinosis (Urbach-Wiethe disease (Urbach, E and Wiethe, C, Li
poidosis cutis mucosae, Virchows Arch. Patholog. Anat., 273, 285-319 (
1929)) is a rare generalized disease with autosomal recessive inherita
nce. It most often involves the skin and mucosal membranes of the aero
digestive tract; but also involves the central nervous system, lung, l
ymph nodes and striated muscles. We present the computed tomography fi
ndings in the cranium and larynx of two siblings with lipoid proteinos
is.