Paragangliomas are tumours of neuroectodermal origin and hormonally active in rare instances. They are very unusual in the paranasal sinuses. We report on the first case--as far as we know--of a recurrent nasal paraganglioma, which started to produce ACTH after a period of ten years and resulted in Cushing's syndrome. Resection of the tumour normalised ACTH and cortisol secretion.