Pyoderma vegetans is a rare disease characterized by the presence of vegetant exudative, pustular and erythematous vesiculobullous plaque usually located in the inguinal area and axillary fold. Etiology of pyoderma vegetans is unknown but it is often associated with bacterial infections in immunocompromised patients. Main histopathological characteristics of pyoderma crops are pseudoepitheliomatous hyperplasia and subepidermal, intraepidermal neutrophilic or eosinophilic microabscesses. It is well known that these lesions are commonly associated with colonic inflammatory disease such as ulcerative colitis and Crohn's disease. Not available standard treatment for pyoderma vegetans, although the use of antibiotic therapy was often used with variable results. Standard first-line therapy is the systemic steroids yet. We perform excision of the lesion of the posterior area of the neck with application of the free split-thickness skin graft after 48 hours postoperatively. In this paper, we present a case of pyoderma vegetans with unusual location without associating colonic lesions and a review of literature related to therapeutic and diagnostic problems of this disease.