This is the case of a 34-year-old woman with Ehlers-Danlos syndrome whose cardiopulmonary manifestations are the following: Prolapse of mitral and tricuspid valves. Aneurysmal dilatation of main arteries without aortic or pulmonary insufficiency. Disturbances in pulmonary function tests and pulmonary arterial hypertension. The diagnosis was verified by skin biopsy and an electron microscopic study. Due to the clinical and histopathological characteristics, we have considered this case to be a non-specified type of the 10 varieties described up to now, and have decided to report it also because of the interesting findings in the hemodynamic and pulmonary function tests.