The extraocular muscles have complex structural and physiological characteristics adapted to meet the motility requirements of fovea-based binocular vision and show unique responses to disease. They are remarkably able to remain intact in muscular dystrophies and motor neuron diseases, but they are preferentially affected in myasthenia gravis, mitochondrial myopathies, oculopharyngeal muscular dystrophy, thyroid associated orbitopathy, and the Miller Fisher syndrome. The reasons behind these unusual properties are only beginning to be revealed.