A new french observation of Lymphomatoid Granulomatosis of Liebow is reported. The case concerns a thirty-five old white woman presenting a cutaneous localisation and a severe pulmonary infection with acute febrile dyspnea and macronodular opacities in chest radiography. Ten months later, encephalic damage led to death. In this observation, the diagnosis is based on skin and chest biopsies showing the characteristic angiocentric and angiodestructive, lymphoreticular granulomatous disease, defined by Liebow. In our knowledge, 152 cases have been reported in North America (5), four cases in Great Britain. This observation is interesting by its rarity, its nosologic discussion it permits.