Severe recurrent attacks of acute febrile neutrophilic dermatosis shortly followed multiple keratoacanthoma in a 43-yaear-old man with myelodysplastic syndrome. Besides the typical clinicopathological findings of the syndrome(fever, leukocytosis and neutrophilic erythematous plaques of the skin), the patient showed orolingual and conjunctival ulceration and severe cutaneous pathergic reactions. Moreover, to the best of our knowledge, multiple keratoacanthoma has not yet been reported with relation to the acute febrile neutrophilic dermatosis. (Korean J Dermatol 1999;37(9) : 1310∼1315)