Turner's Syndrome was first described in 1938 by Henry Turner and has an incidence of 1:3000 live female births. The authors present an unusual case of a 48-year-old woman with late diagnosed Turner's Syndrome. Whose karyotype was (46,X,i(Xq)), associated with a pulmonary emphysema and pulmonary hypertension. The case and the methods of study are presented. Some aspects of this case, namely the hypothesis of an association between the pulmonary emphysema and Turner's Syndrome are discussed.