Endocrine tumor syndromes are individually uncommon or rare. For most medical practitioners, these are unknown or barely known entities and are completely “out of mind.” Collectively, however, patients with these syndromes make up a significant portion of specialist endocrine surgery practice. One of the great rewards of such a practice comes when diligent inquiry into family history and specific investigation for related tumors reveals a patient to be an index 530case with an endocrine tumor syndrome. The consequences for the patient and family are profound, arising from the opportunity to prevent or minimize tumor-related illness.