Immunoglobulin G4-related disease (IgG4-RD) affecting the upper aerodigestive tract is rarely reported in the literature. Most commonly affected sites in the head and neck are salivary glands and the orbit. Here we present the case of a young adult with a laryngeal mass resulted to be an isolated IgG4-RD. Clinical aspects, diagnosis, and treatment are discussed and literature concerning laryngopharynx involvement of IgG4-RD has been reviewed. Authors emphasize that a high index of suspicion and correct diagnostic tools may lead to successful treatment.