As in many specialties, otolaryngology is not without diagnosis surrounded by controversy. This report discusses the entity known as the primary branchial cleft carcinoma, which has been argued by some to be no more than metastatic disease of the unknown primary. Although the disease is rare, the consideration of this diagnosis requires careful thought and analysis by the otolaryngologist, pathologist, and radiation oncologist so that a rational treatment plan can be offered to the patient.