Body integrity identity disorder (BIID), previously called apotemnophilia, is an extremely rare condition where sufferers desire the amputation of a healthy limb because of distress associated with its presence. This paper reviews the medical and philosophical literature on BIID. It proposes an evidenced based and ethically informed approach to its management. Amputation of a healthy limb is an ethically defensible treatment option in BIID and should be offered in some circumstances, but only after clarification of the diagnosis and consideration of (...) other treatment options. (shrink)