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The coincidence of an idiopathic unilateral vocal fold paresis and hereditary hemorrhagic telenagiectasia (HHT) is extremely rare and has not been described in the available literature yet.A 55-year-old female was admitted to hospital due to acute onset of hoarseness, voice fatigue, and effort dyspnea. In the past, the patient was diagnosed with HHT and on admission presented characteristic vascular lesions in the oral cavity. She reported also experiencing a moderate epistaxis at least once per month.The otolaryngological examination (fiberolaryngoscopy, phoniatric examination) revealed unchanged mobility and morphology of the right vocal fold and paresis of the left vocal fold in intermediate position. Computed tomography and magnetic resonance imaging of head, neck, and chest were inconclusive and showed no pathologic findings.The unilateral paresis was treated for 12 months as idiopathic, with extensive rehabilitation. However, no improvement was observed. As a patient suffering from HHT is a challenge for anesthesiologists in terms of general anesthesia, the decision to perform type I thyroplasty (medialization) in local anesthesia was made.There were no complications intraoperatively or in postoperative period. The implemented treatment was successful, as the voice quality improved both in perceptual evaluation (GRBAS scale) and acoustic analysis (F0, jitter, shimmer, NHR).A routine surgical treatment in patients with HHT is a challenge. However, in this case, it was uneventful and successful, thus it can be recommended in other patients with similar background.