An 8-year-old female presented with severe hypernasality and suspected velopharyngeal dysfunction (VPD). Physical examination demonstrated a hypoplastic hemi-velum, tongue atrophy, and deviated uvula. Nasopharyngoscopy demonstrated a significant, asymmetric velopharyngeal gap with adynamic hemi-velum and no lateral pharyngeal wall movement. Given the patient's focal neurologic findings, a magnetic resonance imaging was obtained for further evaluation. Magnetic resonance imaging findings were consistent with Chiari malformation type 1. This case illustrates an unusual presentation of VPD likely secondary to cranial nerve compression and resulting atrophy. Chiari malformation should be considered in the differential diagnosis of noncleft VPD, especially in the setting of palatal asymmetries or other focal neurologic deficits.
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