Superior limbic keratoconjunctivitis (SLK) is characterized by chronic inflammation of the superior limbus and superior bulbar and tarsal conjunctivae. Patients also often have fine punctate staining of the limbus and adjacent area, superior limbic proliferation seen as thickening of the limbal epithelium and surrounding conjunctiva, and occasionally filaments at the superior limbus and upper cornea. SLK frequently presents with ocular irritation, foreign body sensation, and photophobia. SLK can be associated with other ocular and non-ocular conditions, such as thyroid disease. Although the pathogenesis of SLK is still unknown, it is thought to be related to mechanical injury, tear film instability, or an autoimmune/inflammatory etiology. Many patients with SLK can be asymptomatic or have symptoms that resolve or remit spontaneously. For symptomatic SLK, patients are managed medically with treatments such as lubricants, topical anti-inflammatory or immunomodulatory medications, punctal occlusion, and bandage contact lenses. Patients with symptoms refractory to medical management may need surgical interventions. We detail the current literature on the epidemiology, clinical manifestations, associated conditions, histopathology, pathogenesis, and treatment of SLK.