A reliable method for evaluating biopsy specimens of human extraocular muscles is presented to better understand the pathological responses of these highly organized striated muscles. Three muscle fiber types and their distribution are described with morphological and histochemical measurements used commonly for limb muscle. The granular and fine fibers have single end plates and may be comparable to limb-twitch fibers (type 2 and type 1 fibers). The coarse fibers have multiple end plates and may correspond to multiple end plated tonic fibers found in avian and amphibian limb muscles. The fibers of extraocular muscles are arranged in three concentric zones. Because of the zonal arrangement, a complete cross section should be evaluated in diseases of the ocular muscles to estimate any changes in fiber type distribution.