Objectives Axillary artery aneurysms in children are rare and multifactorial. The presenting clinical picture can range from an incidental discovery to threatening symptoms, including loss of extremity function. With the present study we aim to give a comprehensive review of the existing literature focusing on the etiology and management of this rare but threatening pathology. Method A comprehensive review was made in a multi-staged manner. All identified studies have been categorized based on the type of lesions (true or false aneurysm) and their etiologies. Results The treatments differ from the extension of the lesions and from the etiology. Conclusions Open surgery repair with great saphenous vein is still the best long-term approach, but even endovascular, embolization, or conservative management can be considered.