Pseudoaneurysms of the aortic root are rare. A case of prosthetic aortic valve infection progressing from a confined intramural abscess to a ruptured abscess communicating with the aorta and forming a large pseudoaneurysm is described. Additionally, data from all cases and case series, published between 2000 and 2021, was analyzed. A PUBMED search for the keywords "aortic root mycotic aneurysm," "aortic root abscess AND infective endocarditis," and "aortic root mycotic aneurysm AND infective endocarditis" yielded 152 publications (with 157 cases described): Aortic pseudoaneurysm is more common in males (80.9%, n = 127). Mean age is 51 years (4 months-84 years). The most common symptom is fever (68.5%, n = 102). Mean time until diagnosis is 27.2 days. Embolic complications are present in 17.8% (n = 28) at diagnosis. Most cases are due to valvular infections (n = 72 cases, 45.9%). Prior cardiac surgery is documented in 49.0% (n = 77). The mean time interval for developing aortic root abscess following heart surgery is 32.2 months. 22.3% (n = 35) are immunocompromised. Aetiological agents were Staphylococcus sp. (34.1%, n = 47) and Streptococcus sp. (23.2%, n = 32). Mean antimicrobial therapy lasts 58.5 days. Outcome with surgery is superior to medical treatment: overall inpatient mortality 18.5% (n = 27); with surgery 12.2% (n = 15 out of 123 patients), with only medical management 47.8% (n = 11 out of 23 patients). In conclusion, aortic root pseudoaneurysm occurs most commonly in middle-aged male patients. History of prior aortic procedures is commonly present. Correct diagnosis hinges on detailed history, transoesophageal echocardiography, and computed tomography (CT) aorta. Surgery is the preferred therapeutic option.SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12055-023-01580-x.