Tumors of the papilla and ampulla of Vater are rare neoplasms which are usually detected at an early stage due to their symptoms. The accurate preoperative histological diagnosis and staging of ampullary tumors is often difficult and inconclusive, leading to controversy over the adequate treatment of these lesions. Three procedures are currently being used to treat such tumors. Pancreatoduodenectomy (PD) is a procedure with low morbidity and mortality at experienced centers, and is considered the treatment of choice for invasive carcinoma and large benign ampullary lesions with suspicion of malignancy. Transduodenal local excision (TDE) of ampullary tumors is a relatively simple procedure with operative morbidity and mortality rates comparable to PD. TDE is challenged at endoscopic centers by endoscopic snare excision (ESE). Due to technical advances, the safety and outcomes of ESE for ampullary tumors have improved in recent years. ESE and TDE represent adequate methods for treatment of benign tumors and also for small malignant tumors detected at an early stage if the diagnosis and stage have been accurately established preoperatively. Due to the safety of PD and the technical advances of ESE, TDE is reserved for selected patients. Randomized controlled studies are needed to establish the correct indications for PD, TDE, and ESE.