Tuberous Sclerosis Complex (TSC) is easily discernible by a myriad of manifestations, most notably dermatological. It is associated with well known and recognised intra-abdominal tumours like angiomyolipoma of the kidney. However, rarer tumours like pancreatic neuroendocrine tumours can occur in the setting of TSC. A high index of suspicion is necessary to identify and treat these lesions early in their natural course. Early identification augurs well with complete surgical excision and excellent survival.

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Carcinoma occurring the setting of chronic calcific pancreatitis (CCP) are usually multifocal, poorly differentiated, advanced on presentation and associated with poor overall survival. Hence it is imperative that these patients should be identified in the early stages of malignant progression where by curative resection can then be employed. If not, only palliative approaches are feasible in advanced stages. We wish to present our experience of pancreatic carcinoma in the setting of CCP along with a review of literature.

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