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A case of branchiogenic carcinoma is reported herein. The patient was a 59-year-old female who noticed a mass on the left neck and visited our hospital. CT and echography revealed a cystic lesion, and PET disclosed multiple abnormal accumulations on the bilateral cervical lymph nodes and pharynx. First of all, we suspected the tumor as being a malignant lymphoma or metastatic carcinoma. We resected the tumor under general anesthesia for biopsy. The histopathological diagnosis was squamous cell carcinoma arising from a branchiogenic cyst. We examined the whole body and added the biopsies of the epipharynx, palatine and lingual tonsils which revealed no malignancy and no other primary lesions existed. The tumor was therefore diagnosed as a branchiogenic carcinoma. Bilateral radical neck dissection, and postoperative chemotherapy and radiotherapy were performed. The patient has survived until the time of writing for 30 months without recurrence.