Ocular involvement occurs in a significant number of hereditary metabolic disorders (HMDs). Cataract is one of the ocular findings that is frequently encountered. It causes a loss of vision in children which would significantly affect the child`s neuromotor development. The appearance of cataract in HMDs may occur within the first year of life, as well as in other periods during childhood. While cataract is a pathognomonic finding in some HMDs, it is also a faint finding accompanied by other organ involvement that may only be identified by a careful examination. It is very important to recognize cataracts at an early age in order to prevent irreversible amblyopia, early diagnosis and treatment of metabolic diseases that may be related to cataract, to prevent obstacles in the child`s neuromotor development and to intervene rapidly. Taking into account the above considerations, this article discusses cataracts in HMDs in detail.

Keywords: cataract, inherited metabolic disorders, children