Summary: | Abstract Background Heterotopia of pancreatic tissue in the thorax and mediastinum is uncommon, although, a common developmental anomaly in the gastrointestinal tract where the aberrant tissue is a component of gastrointestinal duplication cysts, intralobular pulmonary sequestration or teratomas. Case presentation We report a case of an ectopic pancreas located in a giant mediastinal cyst in a 2-year old girl who presented with severe dyspnoea due to external compression of the intra-thoracic structures, mainly the right main bronchus. Surgical resection was carried out with complete relief of symptoms. The cyst was confirmed histopathologically to contain pancreatic tissue. Literature review showed that this is an uncommon presentation hence the need to report the case. Conclusion Heterotopia of the pancreas although, an uncommon occurrence in the thorax and mediastinum, should be considered when dealing with cystic lesions of the mediastinum and surgery plays an important role in its management. Trial registration JUTH/DCS/ADM/127/XXV/203. Registered 2nd September 2018.
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