Sweet’s syndrome (acute febrile neutrophilic dermatosis) consists of acute onset of painful cutaneous erythematous lesions, mostly found in the upper extremities followed by the head and neck region, particularly in patients with underlying malignancies. We describe the case of a woman in her mid-30s, who was treated for acute myeloid leukaemia and presented with a severe painful and progressive erythematous lesion of the retroauricular skin. Clinical features, laboratory tests, blood cultures and histological biopsy yielded a diagnosis of Sweet’s syndrome. The treatment consisted of oral and topical corticosteroids and her signs and symptoms resolved within 1 week. Although Sweet’s syndrome is uncommon, awareness among otolaryngologists is crucial to ensure a prompt diagnosis, cure and referral to an oncologist (if not already involved) for patients with Sweet’s syndrome in the head and neck area.