Atopic keratoconjunctivitis (AKC) is a chronic allergic inflammatory disease that is at the severe end of a spectrum of allergic conjunctival diseases. AKC can involve the cornea and conjunctiva bilaterally, and at times can lead to visual loss from corneal complications. The classification, histology, ocular examination findings and complications of AKC are described herein, as well as the roles and interactions of inflammatory cells involved. Finally, current treatment options for AKC is reviewed and presented as a stepwise, multidisciplinary approach that involves the allergist/immunologist's medical interventions of topical and systemic immunomodulating agents, as well as the surgical skills of the ophthalmologist.