Congenital malformations or developmental anomalies are terms that denote a deviation from normal body structure, behavior, function, and metabolism, which occurred before birth. Although most anomalies manifest at birth, some of them, especially metabolic and neurological disorders, with the consequent development of structural anomalies, manifest late postnatally. Causes of congenital anomalies are divided into genetic, environmental, multifactorial and unknown. Genetic factors are the most common (about 25%), while only a small number of congenital anomalies are caused by environmental factors (about 12%). Substances that have the potential to induce a structural anatomical anomaly in the fetus are called teratogens. Congenital anomalies can be isolated, involving one or many areas, and multiple, affecting multiple body regions. Isolated anomalies are an etiologically heterogeneous group and may arise due to environmental factors or mutations in a single gene. Multiple anomalies usually have a common cause and involve different, unrelated anatomical areas. According to the clinical significance, isolated congenital anomalies are divided into major and minor anomalies, and multiple anomalies form a combination of multiple major and minor anomalies. Major anomalies cause significant dysfunctions incompatible with life and to function normally require surgery to be corrected. Minor anomalies have no serious clinical significance. Depending on the pathogenesis and the period of occurrence, there are four types of isolated anomalies (malformation, disruption, dysplasia, and deformity) and three types of multiple anomalies (syndrome, sequence, and association). Congenital anomalies of the eye can be defined as developmental disorders that are observed at birth or manifest before the end of development in those species in which development continues shortly after birth. They can be unilateral or bilateral and etiologically different. The list of causes established up until now of congenital anomalies of the eye includes various teratogens, infectious diseases, dietary deficiencies, chromosomal abnormalities, and gene mutations. In most cases, the cause is unknown. Eye abnormalities can occur in the period of early organogenesis as a result of disorders in the formation of the optic vesicle or during its deepening process and the formation of the optic cup. Most anomalies occurring during this period of eye development are associated with neuroectoderm defects, with the exception of lens anomalies, which are surface ectoderm defects. Anomalies that occur after the closure of the fetal eye fissure, during the later embryonic development of the eye, are characterized by disturbances in growth, migration, and differentiation. Anomalies of later development include defects of neuroectoderm, periocular mesenchyme, and superficial ectoderm.