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"Papillary cystadenoma lymphomatosum syndrome" is an unusual clinical constellation that was first described in 1970 as infected adenolymphoma syndrome. A case of papillary cystadenoma lymphomatosum syndrome is presented demonstrating the specific histopathology of the lesion as well as the typical clinical history of all reported papillary cystadenoma lymphomatosum complicated by acute onset of pain and sudden increase in size. Five other cases noted in the literature are compared to complete the clinical and histopathologic review of the entity.
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