As the most severe inherited retinal dystrophies, it is characterized by congenital blindness or severe visual impairment within the first year after birth, nystagmus, sluggish pupillary responses, photophobia, and high hyperopia.[21,22]SH3BGRL2, known as SH3 domain binding glutamate rich protein like 2, belongs to the SH3BGR family. Here, SH3BGR is linked to pathologic nystagmus.