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Acne keloidalis nuchae (AKN) is a rare chronic inflammatory condition most commonly treated medically but in severe, treatment-refractory cases, surgery can be a better solution. In this case report, we present a patient with severe refractory AKN, treated with excision and direct split-thickness skin grafting in combination with negative pressure wound therapy. The patient achieved a satisfactory cosmetic and functional result within a relatively short healing period of six weeks.