Introduction: Pyoderma gangrenosum of the scalp is a extremely rare manifestation of Crohn's Disease in an atypical location. The disease is part of the neutrophilic dematoses. Diagnosis needs to be established histologically. It's longterm outcome is not predictable and pathogenesis is unknown. Potential differential diagnoses are basal cell carcinoma, spinal cell carcinoma, sarcoma, lymphoma as well as cutaneous manifestations of lymphoma. In general, immunosuppressive therapy is regarded as a prerequisite for surgical treatment. Epicrisis: The diagnosis Pyoderma gangrenosum of the parietal scalp was established in an 80 year old female patient who suffered from an acute episode of Crohn's Disease. Further lesions of pyoderma gangrenosum were found at the mons pubis and the external auditory canal. Immunosupressive treatment with steroids, azathioprine and infliximab resulted in remission of the diarrhea with bloody and mucous constituents and progression of the Pyoderma gangrenosum could be prevented. Surgical necrosectomy and decortication at a maximal range of 13 x 10 cm of the exposed scull was performed. After sufficient granulation, supported by vacuum assisted closure therapy, the defect was covered by a split skin graft taken from the right thigh. The patient was followed up for 404 days from first examination and is being continually treated with low dose steroids and azathioprine. So far, there are no signs of relapse. Results: The importance of immunosusppressive treatment of Pyoderma gangrenosum as a precondition for successive surgery was evinced in this course of disease. Vacuum assisted closure therapy seems to be an effective aid in supporting granulation and preventing wound infection. Granaulation tissue originating in the cancellous bone of the skull provided a sufficient basis for alimentation of split skin graft. Keywords: Crohn's Disaese, Pyoderma gangrenosum, exposed skull, immunosupressive therapie, steroids, azathioprin, infliximab, necrosectomy, decortication, granulation, vaccum assisted therapy, split skin graft