Background: Axillary artery aneurysms are rare conditions, and their causes are various. They can determine severe complications, so the treatment is extremely important. Methods: We report the case of a young man affected by a saccular axillary artery aneurysm associated with intramuscular arteriovenous malformation, without symptoms except for the presence of a pulsatile mass. Duplex scan and computed tomography scan have been essential for a correct diagnosis and planning of the treatment. At first, the patient was submitted to coil embolization of an efferent vessel, and then he was treated surgically through ligation and detachment of the aneurysm and replacement of part of the axillary artery with a Dacron graft (Vascutek, Inchinnan, Renfrewshire, Scotland, UK). Results: Follow-up at 1 and 6 months revealed normal patency of the axillary arterty and the prosthetic graft with complete exclusion and thrombosis of the aneurysm sac.No sensitive nor motor deficit were observed. Conclusions: Aneurysms of the axillary artery associated with intramuscular arteriovenous malformations are very rare, but have to be suspected. The treatment is challenging and can be surgical, endovascular, or hybrid, based on the patient's conditions and aneurysm's anatomical features.