The molecular study consisted of a next-generation sequencing panel containing 14 genes associated with Usher syndrome: MYO7A, USHC1, CDH23, PCDH15, USHG1, CIB2, USH2A, ADGRV1, WHRN, CLRN1, HARS, PDZD7, CEP250, C2orf71.We present the case of a 13-year-old girl from a consanguineous Colombian family diagnosed with Usher syndrome type 1G. The gene discussed is PDZD7; the disease is Usher syndrome.