Acinar cell carcinoma of the pancreas is rare, accounting for less than 1% of carcinomas arising in the exocrine pancreas. Patients are typically between the fifth and seventh decade of life and show a 2:1 male predominance. Symptoms tend to be nonspecific, and approximately 50% of the patients have metastases at the time of presentation. There have been recent case reports of acinar cell carcinoma showing both intraductal and/or papillary patterns of growth that could potentially be mistaken for intraductal neoplasia. The cases reported up to date presented as solitary nodules. The authors describe the first case of acinar cell carcinoma with both intraductal and tubuloglandular growth diffusely involving the pancreas. In addition, the authors review the published cases reporting acinar cell carcinoma with intraductal and/or papillary growth patterns.