High myopia (HM) is defined as refractive error above -6.0 D (-8.0 diopters) with axial eyeball length above 26 mm, and is connected with the process of excessive myopisation. HM is not a homogenous disease. HM is considered to be inherited in 3 different patterns: dominant, autosomal recessive, and X-linked. Many genetic mutations linked to the development of HM have been described, including high grade myopia, and MYP1-16; different patterns of inheritance may reflect different types of HM. Over 150 genetic syndromes are associated with HM. The clinical state of the HM eyeball may also depend on environmental risk factors influencing the progression of refractive error. The complexity of etiopathogenesis makes it difficult to distinguish to what extent the development of HM is related to genetics versus exposure to environmental factors. HM remains a leading cause of visual loss. HM and its complications are considered to be one of the most significant causes of blindness and visual impairment in young, professionally active people, becoming an important social problem. Contemporary data concerning genetic factors, family aggregation, and epidemiological data of visual impairment connected with HM are presented in this paper. Therapeutic options for this refractive error are presented as well.