The first reported case of a patient with chronic lymphocytic leukemia, who 14 years after diagnosis developed bilateral symmetrical ear lobe swelling secondary to lymphocytic infiltrates and its management, is presented. The various otologic and specific skin manifestations of leukemia are discussed and the literature reviewed. The temporal bone findings take the form of leukemic infiltrations, inflammation, and hemorrhage. These may present as a red or thickened tympanic membrane, hemotympanum, exudates in the middle ear, acute otitis media, mastoiditis, conductive or sensori-neural hearing loss, dizziness, vertigo or facial paralysis. The specific skin manifestations take the form of nodules, papules, infiltrations, plaques, ulcerations and exfoliative erythroderma. These may present as skin lesions most commonly in the head and neck area, rarely presenting in the auricle and ear canal.