Background: Epidermoid cyst in intrapancreatic accessory spleen (ECIPAS) is a rare benign lesion that may mimic pancreatic cystic neoplasms. Case Report: A 46-year-old male presented with abdominal pain, obstructive jaundice, and pruritus. Following diagnosis of periampullary adenocarcinoma, Whipple’s pancreaticoduodenectomy was performed. Histopathology revealed moderately differentiated adenocarcinoma and an incidental pancreatic cyst lined by stratified squamous epithelium, surrounded by lymphoid tissue with splenic sinusoids highlighted by CD8 immunohistochemistry, confirming ECIPAS. Conclusion: This rare coexistence highlights the importance of thorough histopathological examination and CD8 immunohistochemistry in diagnosing ECIPAS in pancreatic resection specimens.