Ampullary carcinoma belongs to the cluster of periampullary cancers and is a rare, but increasing form of gastrointestinal malignancy. Due to the location of the tumour, occurrence of biliary obstruction is common. Symptoms due to the compression of the biliary tract facilitate early diagnosis, evoking a better prognosis. Adenomas of the ampulla of Vater and major duodenal papilla are precursor lesions and possess a risk of 30-40% to progress into a malignancy. Therefore, en-bloc resection is warranted for all ampullary adenomas. Endoscopic papillectomy is aggravated by extension into the pancreatic duct or common bile duct. Surgical resection is indicated whenever endoscopic resection is incomplete or infiltrative growth is suspected. Transduodenal ampullectomy is an alternative to extensive oncological resection in the absence of malignancy. Pancreatoduodenectomy (or Whipple procedure) with systemic lymphadenectomy and mesopancreas excision is the standard procedure of all ampullary carcinomas and incompletely excised adenomas by minimally invasive procedures. The indication for extensive surgical resection includes suspicion of infiltration in endoscopic ultrasound or evidence of malignancy in frozen section during transduodenal ampullectomy. Negative prognostic indicators are implicated by the pancreatobiliary subtype, lymph node metastases and perineural invasion. Differentiation of the different histopathological subtypes thereby increases in clinical relevance. Evidence based guidelines for the clinical practice of neoadjuvant and adjuvant treatment for ampullary carcinoma have yet to be defined. According to the literature available, patients with the pancreatobiliary subtyp or association with other negative prognostic factors seem to benefit from systemic therapy. Further studies are warranted.