This is a case report of a 76-year-old woman, who had Brooke-Spiegler syndrome (BSS) and presented with multiple confluent tumours of the scalp. An MRI showed an intracranial invasion. Multiple excisions were made due to recurrence of the intracranial tumour, and reconstruction was achieved with free flaps and skin grafts. BSS is caused by a mutation in CYLD, a tumour suppressor gene located on chromosome 16q12-q13. Surgical excision is often not a curative treatment, and the recurrence rate is 35%. Quality of life is significantly affected with regards to cosmetic appearance, painful tumours and multiple surgical treatments.