Relapsing acute febrile neutrophilic dermatosis ( AFND ) is reported in a patient with essential thrombocythemia representing the first case of this kind described in the literature. The patient presented with fever, malaise, neutrophilic leukocytosis, dysproteinemia, and coalescing bluish-red painful plaques and papules at palms, forehead, lid, forearm, and thigh. There was a dramatic response to steroids, or indomethacin, with no scarring. AFND was preceded by sore throat and an upper respiratory infection and was accompanied by conjunctivitis and arthritis of knees and ankle-joint. The association of AFND with essential thrombocythemia and with other neoplastic disorders is discussed.