Vernal keratoconjunctivitis (VKC) is a bilateral, usually seasonally recurrent inflammation of the conjunctiva. Clinically characteristic findings are tarsal giant conjunctival papillae (> 1 mm) and/or limbal gelatinous changes (Trantas dots). The underlying etiology and pathophysiology of VKC remains unclear; however, clinical findings and immunohistochemical studies suggest a complex, both IgE-dependent and IgE-independent immune-mediated etiology. Several predisposing conditions include endocrine, genetic, neurogenic, environmental and socioeconomic risk factors. Mast cell stabilizers, antihistamines and topical corticosteroids are often used during acute flare-ups in VKC; however this approach is unsatisfactory for controlling severe cases and avoiding recurrences. Immunomodulatory agents, such as cyclosporin A and tacrolimus are promising alternative agents for long-term management. In most children the clinical course of VKC is self-limiting and may disappear following puberty; however, some VKC patients will face sight-threatening complications which are mainly due to corneal involvement and iatrogenic damage caused by prolonged corticosteroid treatment.