Introduction: Mature cystic teratoma originating in the pancreas is very unusual, often observed as an incidental finding during routine examinations or recognized perioperatively as the patients present with very unspecific clinical symptoms. The confirmatory diagnosis of a pancreatic cystic teratoma is generally made by histopathology after surgical excision. So, the preoperative diagnosis is very challenging, especially differentiation from the other pancreatic pathologies. Patient concerns: A 23-year-old woman was admitted to our hospital with a complaint of mild grade periumbilical abdominal pain. A pancreatic mass was revealed on a preliminary abdominal ultrasound examination. Her medical history was unremarkable with no long-standing illness or malignancy. Diagnosis: Mature cystic teratoma in the head of the pancreas. Interventions: Roux-enY choledochojejunostomy with gastrojejunostomy was performed, excising the tumor from the pancreatic head. Outcomes: The postoperative course was uneventful; the patient was asymptomatic and has no evidence of recurrence on a 2-year follow up. Conclusions: Pancreatic cystic teratoma is a benign, well-differentiated, and extremely rare congenital tumor. MRI is the choice of imaging modality and phase-GRE or fat suppression is the best technique for pre-operative diagnosis.