Background: Worster-Drought syndrome (WDS) is a congenital, pseudobulbar paresis. Patients show oromotor apraxia causing impaired speech, drooling, dysphagia and varying degrees of cognitive impairment. Familial cases are reported although causative genes have not been identified. LINS mutations have recently been reported in patients with severe cognitive and language impairment. Methods: The proband was diagnosed with WDS at 8 years old because of longstanding drooling, dysphagia and impaired tongue movement. At 14 years old, he remains aphonic, using sign language and typing on a smart-tablet to communicate. Neurological examination including facial and extraocular movement was otherwise unremarkable. MRI brain revealed no heterotopia or atrophy. Results: An expanded intellectual disability panel at GeneDx identified nonsense mutations in LINS alleles: c.1096; p.Glu366X and c.1178 T>G, p.Lys393X. Neuropsychological testing at 14 years old noted nonverbal reasoning skills at 5 year old level with relative sparing of his receptive vocabulary and visual attention. Compared to prior testing at 9 years his receptive language improved from a 6 year old to an 8.5 year old level. Conclusions: Nonsense mutations of LINS have been identified in a patient with WDS. Despite his severe and persistent aphonia, improvements in receptive language were observed with global intellectual functioning better than expected.