Henoch-Schönlein purpura (HSP)/ IgA vasculitis (IgAV) is the most common form of systemic vasculitis in children and often involves the skin, gastrointestinal tract, joints, and kidneys, though cardiac involvement rarely occurs. We report on a 6-year-old male child with HSP/IgAV who had renal and cardiac involvement at the initial stage of the disease and in whom we found an extremely rare coronary artery aneurysm. After administration of glucocorticoid combined with mycophenolate mofetil, the renal involvement improved, but the coronary artery aneurysm remained. Pursuant to this case, we retrieved information on other cases of HSP/IgAV complicated with cardiac involvement from the PubMed database, and excluded cases of cardiac involvement accompanied by Kawasaki disease, polyarteritis nodosa, rheumatic fever, Takayasu arteritis, systemic lupus erythematosus, poststreptococcal glomerulonephritis, or sepsis. We then analyzed gender, age, cardiac involvement, renal involvement, treatment, and prognoses. To date, 24 cases of HSP/IgAV complicated with cardiac involvement have been reported. Among them, there were 22 male and 2 female patients, with the onset age ranging from 3 to 71 years old. A total of 10 children (including the child we examined) and 14 adults were identified, and 17 patients (70.8%) had HSP/IgAV complicated with renal involvement. The majority of patients were treated with glucocorticoid and/or immunosuppressants or biological agents, 4 patients died (16.7%), 8 patients were completely relieved (33.3%), and 3 patients had unknown prognoses. This article suggests that HSP/IgAV complicated with cardiac involvement may result in a poor prognosis and early treatment may therefore be essential. Our case revealed that glucocorticoid does not prevent the occurrence of renal and cardiac involvement in HSP/IgAV patients. If HSP/IgAV is complicated with coronary artery dilation, the therapeutic effect of glucocorticoid combined with immunosuppressants is not satisfactory, and early administration of biological agents or IVIG may be an effective therapeutic regimen.