Development of modern surgical techniques is associated with the need for a thorough knowledge of surgical anatomy and, in the case of ophthalmologic surgery, also functional aspects of extraocular muscles. Thus, the leading idea of this review was to summarize the most recent findings regarding the normal anatomy and anomalies of the extraocular rectus muscles (ERMs). Particular attention was paid to the presentation of detailed and structured data on the gross anatomy of the ERMs, including their attachments, anatomical relationships, vascularization, and innervation. This issue of ERMs innervation was presented in detail, considering the research that has recently been carried out on human material using advanced anatomical techniques such as Sihler’s technique of the nerves staining. The text was supplemented with a carefully selected graphic material (including anatomical specimens prepared specially for the purpose of this review) and discussion of the clinical cases and practical significance of the presented issues.