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Context: Solitary true cysts of the pancreas in adults are extremely rare, and only few cases have been reported in the literature. The etiology and natural history of these lesions remain unknown, and treatment is not standardized. We describe an additional resected case. Case reports: A 55-year-old female who presented to us with a history of heaviness and pain in right upper abdomen for 1 year. CT scan of abdomen showed a large unilocular cyst in the region of the head of the pancreas. The patient underwent pancreatico-duodenectomy with binding pancreaticojejunostomy. The histology of cyst revealed true unilocular pancreatic cyst lined by cuboidal epithelium. She is well after 3 years of follow-up. Conclusions: A preoperative work-up alone does not always allow an accurate diagnosis, but it is useful in determining lesion characteristics and guiding therapeutic decision-making. When surgery is indicated, a limited resection is warranted in most cases. [ABSTRACT FROM AUTHOR]