Paget’s disease is a rare type of breast cancer characterized by the proliferation of large cells with clear cytoplasm in the epidermis. Paget’s disease occurring in regions other than the breast, such as in the external genitalia and/or perianal region, is known as extramammary Paget’s disease. Herein, we present the case of a 77-year-old man with extramammary Paget’s disease of the larynx. The patient was referred to our hospital with a mass on the right side of the neck. Endoscopic examination of the larynx showed a smooth mass covered by intact mucosa involving the right aryepiglottic fold. Biopsy of the laryngeal tumor led to the diagnosis of extramammary Paget’s disease. Radiotherapy was administered at a total dose of 66 Gy, in view of the patient’s age, likelihood of complications, and requirement of larynx-preservation. Following radiotherapy, the laryngeal tumor and the cervical lymph node metastasis disappeared and the patient remains alive, more than 2 years after the treatment. Aside from surgery, radiotherapy may also be an effective treatment option for ectopic extramammary Paget’s disease of the larynx.
