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.