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Epidermoid cysts are rare lesions that can occur anywhere in the body. They are associated with elevated serum levels of CA 19-9. The spleen represents the most common site of intra-abdominal localisation. Only two cases of diaphragmatic epidermoid cyst are reported in the literature. We present the case of a 61-year-old woman with a small suprasplenic subdiaphragmatic cyst discovered during the investigation of left flank pain. The establishment of an adequate diagnosis was challenging due to the difficulty in specifying the exact localisation of the cyst, the extremely elevated CA 19-9 level of 19,000 and the high uptake on 18-fluoro-2-deoxy-D-glucose positron emission tomography. The definitive diagnosis followed complete surgical excision. Intra-abdominal epidermoid cysts are usually discovered incidentally on imaging for another reason. The cyst is lined by squamous epithelium responsible for the secretion of CA 19-9. The elevation of serum CA 19-9 is due to small rupture or increased intraluminal pressure followed by diffusion to the bloodstream. Surgery with en-bloc resection represents the optimal treatment to avoid any risk of recurrence. The definitive diagnosis is established by demonstrating positive immunohistopathological staining of epithelial cell to CA 19.9.

A rare presentation of small diaphragmatic epidermoid cyst with extremely elevated serum CA19-9 level