This case report describes the diagnosis and therapy of a patient with a 40-year history of multiple potato-like tumours growing in the head and neck region. The tumour proved to be a cylindroma, associated with calvarian defects. Further facial tumours were diagnosed as trichoepitheliomas. This association of findings was pathognomonic for Brooke-Spiegler's syndrome. Complete work-up of the resection specimen excluded any malignant transformation of the tumour. A long lasting history of cylindroma and evidence for bone destruction, not associated with a malignant transformation, is extremely rare in Brooke-Spiegler's syndrome.
