This report describes a rare benign cystic lesion of the pancreas known as acinar cell cystadenoma. There are 12 previously reported cases, the youngest patient from which was age 16. We report the youngest case to date, occurring in a 9-year-old boy. The pancreatic lesion was found incidentally and confirmed by laparoscopic biopsy. A pseudocyst formed, which was drained endoscopically. Given that the lesion was benign and the patient was asymptomatic, no further intervention was recommended. Complete resection would require a total pancreatectomy, with attendant morbidity, and review of the literature suggests a benign course. In asymptomatic cases, we recommend biopsy for histologic diagnosis, with close follow-up and without further surgical intervention.
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