Pyoderma gangrenosum (PG) is an ulcerating, neutrophilic dermatosis of unknown etiology. Clinical appearance is characterized by sudden onset of sterile pustules that rapidly develop into very painful ulcerations with violaceous, undermined borders. Due to the lack of specific diagnostic and therapeutic markers, PG is a diagnosis of exclusion. An association with further diseases such as chronic inflammatory bowel disease, rheumatoid arthritis, diabetes, neoplasms, or metabolic syndrome exists in over 50 % of cases. Treatment of PG consists of suppression of inflammatory disease activity, treatment of associated morbidities, promotion of wound healing, and pain relief. Recommended first-line treatment consists of systemic glucocorticosteroids and additional or alternatively cyclosporine. A controlled trial on infliximab in PG points to efficacy of TNF-alpha antagonists. Due to chronicity, frequent recurrences, and often complicated courses, treatment with good tolerability is important.