Maturity Onset Diabetes of Young (MODY) is a hereditary form of diabetes mellitus and is caused due to mutations in autosomal dominant gene and is referred to as Monogenic diabetes. It may be confuses to be a disease Latent Autoimmune Diabetes of Adult (LADA). But LADA has a slower progression to insulin dependence as age progresses while MODY has childhood onset and occurs later in life. MODY variant forms include MODY 2 and 3 which are most common forms. Children and adolescents with MODY may have fasting hyperglycemia at diagnosis and these patient presents with typical microangiopathic and macroangiopathic complications with similar degree of hyperglycemia In retrospect we can now recognize that this category covered a heterogeneous collection of disorders which included cases of dominantly inherited diabetes, still call MODY today. As in 20th century the concept and understanding of MODY has become refined and debatable, hence the pathophysiology and management has been explored through literature in this review.