Composite paragangliomas are rare with less than 20 cases documented in the abdomen, retroperitoneum, and urinary bladder. In this article, we report the first case of composite paraganglioma in the head and neck presenting as a soft tissue mass in the neck adjacent to the carotid artery in a 50-year-old woman. We discuss the clinicopathologic findings and genetic implications, and we review the literature of this rare entity.