Cutaneous amyloidosis and multiple endocrine neoplasia 2A (MEN 2A) have been previously reported in several families. A genetic linkage of both disorders has recently been described. Notalgia paraesthetica has been suggested to be involved in the aetiology of amyloidosis in such patients. We report such an association in a 48-year-old woman with several relatives suffering from MEN 2A. Treatment with topical capsaicin, which has been shown to be effective in notalgia paraesthetica, was not useful in our patient.