In this review, we will discuss the structure and functions of the oBRB, and how its disruption contributes to the pathogenesis of a variety of ocular conditions including diabetic retinopathy (DR), age related macular degeneration (AMD), central serous chorioretinopathy (CSCR), Sorsby’s fundus dystrophy, Retinitis Pigmentosa (RP), and conditions associated with mutations in CLDN-19, the gene encoding for the TJ protein claudin-19. Here, CLDN19 is linked to Sorsby's fundus dystrophy.