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Background Patients with Darier disease often present with staphylococcal skin infections and are at risk for complications when they undergo cardiothoracic surgery, such as acute aortic dissection repair. Case summary A 39-year-old woman with hypertension and Darier disease suffered an acute type A aortic dissection, requiring emergency operation with a Dacron graft. Twenty-five days post-operatively, she developed pneumonia and staph hominis was isolated in blood cultures and Bronchoalveolar Lavage. Following completion of antibiotics, multiple relapses occurred during a 6-month period, each time treated with appropriate antibiotic therapy. An 18F-fluorodeoxyglucose positron emission tomography computerized tomography showed persistent graft uptake and re-operation was performed. At 22 months of follow-up, the patient remains asymptomatic and the 18F-FDG PET/CT shows significant reduction in FDG uptake. Discussion Graft infection is a rare but serious complication. Antibiotic therapy is often inadequate and re-operation is needed. As staphylococcal skin infections often occur in patients with Darier disease, adequate preprocedural skin preparation and sterilization are very important in these patients.