The intent of this article is to act as a resource for helping to determine the cause of cataracts based on the lens appearance, age of onset and systemic findings. Children with cataracts, especially when bilateral, require a comprehensive history and ophthalmic examination with physician awareness toward other organ system involvement. A basic assessment of facial, skeletal, genitourinary, gastrointestinal and integumentary abnormalities is beneficial. In this review, there are numerous tables that are to act as a resource in developing a differential diagnosis and guide further systemic and genetic evaluation.