Proteus syndrome is a rare and highly variable hamartomatous syndrome that can affect multiple organ systems. It is characterized by hyperplasia of connective tissue, vascular malformations, epidermal naevi and hyperostosis. Most of the cases present to plastic and orthopaedic surgeons. Otolaryngologic presentations are minimal. We report such a rare case, which presented with a huge unilateral tonsillar mass causing dysphagia.