We report a case of a 20-year-old-man who was admitted to our hospital through the emergency room for a swelling of the right thigh, which had appeared spontaneously without fever. The physical exam was highly suggestive of a vascular mass. The arterial CT scan of the right lower limb revealed a pseudo-aneurysm of a branch of the deep right femoral artery. The diagnosis of vascular type of Ehlers Danlos syndrome was established on clinical criteria. The successful management was surgical by the excision of the pseudoaneurysm and the ligation of the feeding branch that supplied the pseudoaneurysm. The postsurgical follow-up was uneventful and the clinical course was smooth with no further complications.
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