Introduction: Otopalatodigital syndrome type 1 is a rare pathology with a high rate of neonatal deaths and stillbirths. Male individuals present a milder phenotype compatible with life. Its manifestations include hearing loss, cleft palate, oligohypodontia, short fingers and square-shaped distal phalanges, hypertelorism, reduced range of joint motion, and preserved intelligence. Objective: To describe the findings of the orofacial myofunctional phonoaudiological and audiological evaluation and the clinical and instrumental otolaryngological evaluation by nasopharyngoscopy of an individual with otopalatodigital syndrome type 1. Resumed report: At the phonoaudiological evaluation he presented alterations of lip and tongue mobility, atypical swallowing, speech with phonetic alterations of the distortion type on phone [r], sigmatism on phone [s] and phone {S}, interdentalization on phones [t,d,n], besides obrigatory velopharyngeal dysfunction disorders such as nasal air emission, low intraoral airway pressure and mild hypernasality and compensatory articulation of the pharyngeal fricative type on phone [f]. Audiometry revealed mild bilateral conductive hearing loss on the right and moderate on the left. During the otorhinolaryngological evaluation, through nasofibroscopy we found absence of a vomer and sagittal-type velopharyngeal closure with a small gap during repetition of phrases with plosive and fricative phones and spontaneous speech, and complete closure during therapeutic testing for plosive phones. Conclusion: The individual with otopalatodigital syndrome type 1 has orofacial myofunctional alterations, conductive hearing loss and velopharyngeal dysfunction. Speech rehabilitation and follow-up with otorhinolaryngologist are indicated. [ABSTRACT FROM AUTHOR]