Connective tissue disorders (CTDs) involve multiple organ systems and may have a significant impact on the overall health and quality of life of the affected individuals. The present paper aims to review the current knowledge on the laryngeal manifestations of CDTs, and describe the available diagnostic and treatment options. Systematic literature review in Medline and other database sources. Information from related books was also included. Prospective controlled, double-blind prospective, prospective, and transversal cohort studies, case series, case reports, systematic reviews, and consensus papers. Laryngeal involvement mostly occurs in rheumatoid arthritis (13-75% of patients). It is not uncommon in active and progressive clinical course, though can also occur in silent or inactive CDTs. The crico-arytenoid joint is the most commonly affected site. Common symptoms include throat pain, dyphonia and hoarseness. Careful clinical assessment of the larynx by flexible naso-endoscopy, video-stroboscopy, or direct laryngoscopy, and appropriate imaging are required for pertinent patient management. Stridor is a sign of a life-threatening condition, and may require prompt surgical intervention. However, mild symptomatology may mislead clinicians, and the related diagnosis may be significantly delayed. The current evidence as identified in the present study suggest that laryngeal manifestations of CDTs are often underdiagnosed, due to a range of non- specific symptoms. A multidisciplinary team approach with ENT input is necessary to improve the overall patient management.