Xanthoma disseminatum (XD) is a rare mucocutaneous xanthomatosis classified
as a benign form of non-langerhans' cell histiocytosis. The case history i
s presented of a 61 year old woman with XD who developed dyspnoea and spiro
metric features of airflow obstruction. Bronchoscopy and computed tomograph
y confirmed involvement of the large and medium sized bronchi and she subse
quently died from acute respiratory failure. The post-mortem findings and t
he importance of respiratory tract disease in this unusual condition are di
scussed.