Glucagonoma is an uncommon, challenging but treatable disease with varied manifestations. Despite its predominantly malignant nature, prolonged symptom-free survival can be achieved using a targeted combination of surgery, hepatic artery embolization and somatostatin analogues. Given the difficult management issues, an initial assessment in an experienced tertiary referral centre may also be of benefit. This chapter has looked at the long-term follow-up of 18 such patients over a 25-year period. Given the rarity of the tumour, the numbers are small, but valuable lessons can be learnt from the study in the clinical management of these patients.