Dendritic fibromyxolipoma is a rare and distinctive soft tissue neoplasm that is considered by many authors as a variant of spindle cell lipoma and characterized by the presence of dendritic cytoplasmic processes, plexiform vascular pattern, and keloidal collagen. It has never been reported in the larynx and hypopharynx. Its rarity and the potential to mistake it as a more clinically aggressive myxoid soft tissue neoplasm highlight the importance of its recognition. Here, a case of a dendritic fibromyxolipoma of the pyriform sinus in a 38-year-old male who presented with dysphagia, change of voice, and stridor is reported. A review of the literature, including histopathologic features and differential diagnosis, is also included.