The authors report a 64-year-old female with Brooke-Spiegler syndrome who 
presented with multiple cutaneous nodules and tumors mostly involving the scalp. 
Histopathological examination of one of the lesions located in a periauricular 
area revealed a typical cylindroma. In some neoplastic nodules ductal 
differentiation and occasional bilayered glands composed of the dark abluminal 
basal/myoepithelial cells and luminal mucinous cells might be recognized. 
Apocrine secretion was focally noted. Molecular biologic study of the CYLD gene 
performed from the peripheral blood identified a novel splice site c.2041+1 G>T 
mutation. This new germline mutation in the CYLD gene of a Slovak patient with 
Brooke-Spiegler syndrome extends the catalogue of known CYLD germline mutations 
in this condition.