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Splenic vessel aneurysm is often a casual pathology during strumental imaging or laparotomy. The first cause for the formation of the aneurysm is a vessel primitive degenerative pathology of the medium wall, or idiopathic cystic medionecrosis, that to develop in aneurysmatic dissection. In the early diagnosis, in the selective patients, resulted indicate an endovascular theraphy, while in emergency the laparotomy is a gold strandard for reduction morbility and mortality. In this paper, the Autors has described a rare case of rupture of the lienal vessel aneurysm in the Erdheim illness, and has reported clinical characters, indications and procedures for a better treatment.