A 48-year-old female with a history of accentuated dyspnea, pleural thickening in anteromedial portion with left patchy parenchymal shadowing invading adjoining parasternal structure of the rib cage, presented 13 months later marked superclavicular, anterior mediastinic and parahilar left lymphadenopathy. Open surgical biopsies on the pleural lesion invading the hypodermic tissues of parasternal region showed morphological and immunocytochemical patterns of Langerhans' cell histiocytosis (LCH). 13 months later the superclavicular lymph node biopsy diagnosed Hodgkin's lymphoma (HD), mixed cellularity type II, stage AE. On a total of 29 cases with association of LCH and HD, the described case in the second case that shows morphologically demonstrated LCh with subsequent development of HD. It is postulated that the development of HD in a patient with LCH, might represent malignant evolution of this hyperplastic process.