Pyoderma gangrenosum is an inflammatory dermatosis of uncertain etiology, often associated with chronic inflammatory bowel or rheumatoid disease. It predominantly affects the skin. A systemic organ involvement caused by aseptic neutrophilic abscesses, however, fundamentally influences the prognosis.A patient with idiopathic pyoderma gangrenosum insufficiently responded to a conventional immune suppressive therapy and the disease proceeded aggressively. During treatment with Infliximab, partial remission was achieved intermediately. However, a recurring endocarditis, led to a lethal outcome of this patient.