Porphyria cutanea tarda (PCT) in women may be associated with distressing increased growth of vellus or androgen-influenced hair. Hirsutism may persist after clinical and biochemical remission of the PCT. For patients with persisting hirsutism, oestrogen therapy alone or in combination with cyproterone acetate is contraindicated as further disease activity may be provoked. We report the beneficial effect of spironolactone in a patient with PCT-associated hirsutism.