This special issue focuses on the current approaches for diagnosis, evaluation, and management of autoimmune diseases of the anterior segment of the eye, which range from immune keratoconjunctivitis to anterior uveitis. Immune diseases of the anterior segment of the eye may be caused by several local or systemic conditions and may present in a wide range of diseases including dry eye syndrome, ocular cicatricial pemphigoid (OCP), graft versus host disease (GVHD), and some forms of anterior uveitis often associated with systemic autoimmune diseases such as ankylosing spondylitis.