Pseudocoarctation of the aorta is an infrequent anomaly caused by the elongation and the congenital kinking of the aorta. We present a case of pseudocoarctation of the aorta with saccular aneurysm complicated with a large pseudoaneurysm. Both, magnetic resonance and aortography showed an aneurysm of the aortic isthmus that extended/progressed as pseudoaneurysm towards the neck. The patient was asymptomatic but due to the high risk of rupture, she underwent surgical reparation. The revision of the literature and our experience with this case show that pseudocoarctation of the aorta is not always a benign pathology and that surgical reparation should be performed in all those cases that are complicated by aneurysm.