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Background and Objective: Although cleft palate is the most common cause of velopharyngeal dysfunction (VPD), other disorders can also cause hypernasality and/or nasal emission. Velopharyngeal incompetence (VPI) is one of the VPD. VPI is usually associated with a cleft palate but it can occur without a cleft palate. Congenital myopathy (CM) is a muscular disorder that recognizes early onset of the disease, muscle weakness, hypotonia and delayed motor development. Yet do not specify the effect of VPI due to CM, on speech. The purpose of this paper was to describe a case of VPI as one of the main clinical manifestations in a patient with a congenital myopathy. Case presentation: The patient was a four years old female. There was no family history of cleft palate or lip. The only complaint of the family was unclear speech and her hypernasality. After perceptual assessment and diagnosis of severe hypernasality, endoscopy was performed. Endoscopy showed that in the absence of structural abnormalities, soft palate and pharynx walls were moveless. there was a medium gap too. Discussion: Patient speech errors in high-pressure consonants, were severe hypernasality, posterior-nasal fricative and nasal emission. These errors are special features of cleft palate, but in device evaluation, she had no obvious or even submucosal cleft palate. With reference to the neurologist, specify the VPI symptoms, resulting from congenital myopathy. The report emphasizes the need to suspect to a neuromuscular disease when a VPI is diagnosed and there is no evidence of an upper abdominal ulcer and abdominal endoscopic cleft. [ABSTRACT FROM AUTHOR]