Background: Colloid carcinoma of the pancreas is a rare type of pancreatic cancer that has a more indolent course and superior long-term survival compared to ductal adenocarcinoma. There is a dearth of literature describing this diagnosis due to its rarity and its only recent recognition as a distinct clinical entity. We present two cases of patients with colloid carcinoma and discuss the presentation and management of this disease. Case Presentation: A 58-year-old man with repeated bouts of pancreatitis and a 72-year-old woman with symptoms of pancreatic exocrine and endocrine insufficiency were both found to have cystic masses in the head of the pancreas. Both were identified as having at least mixed main duct/side branch intraductal papillary mucinous neoplasms (IPMNs) on appropriate workup with additional imaging and endoscopy. Pancreaticoduodenectomy was recommended. Both patients, however, were noted to have high-grade dysplasia at the resection margin intraoperatively on frozen section, and thus, total pancreatectomies were performed. Final pathology in each case demonstrated colloid carcinoma with no nodal spread of disease. The patients recovered well. Adjuvant chemotherapy was recommended. Conclusion: Colloid carcinoma of the pancreas is a rare pathologic diagnosis and is frequently associated with IPMN. Colloid carcinomas tend to present at earlier stages than do ductal adenocarcinomas and are known to have improved long-term survival. Surgical and systemic options for treatment parallel that of ductal adenocarcinoma due to the rarity of the diagnosis and the lack of trials assessing therapy for this specific diagnosis.