Normal weight obesity (NWO) is defined as having a normal body mass index (BMI), but a high body fat mass. There is growing interest in individuals with NWO, which is an underdiagnosed and understudied group, because of their increased risk for cardiometabolic morbidity and mortality. In this review, we summarized the definition, prevalence, etiology, pathophysiology, and cardiovascular outcomes seen in NWO. We have also summarized the available literature on interventions for NWO. There is a wide variation in the body fat percent cutoffs used to diagnose excess body fat. Hence, the prevalence rates of NWO vary between different populations and studies. It is estimated that about 30 million Americans have NWO and the worldwide prevalence ranges from 4.5% to 22%. Genetics, diet, and physical activity are related to NWO. However, etiological factors are not clear. Changes in body composition, inflammation, oxidative stress are present in NWO in comparison to normal weight lean (NWL) who have a normal BMI and normal body fat amount. Furthermore, cardiometabolic changes are observed and some are subclinical. Thus, screening for NWO will enhance the primary prevention of cardiovascular disease. Due to the use of various body fat percent cutoffs and methods to measure body fat, it is challenging to compare between studies. Researchers working in this field should ideally work towards developing standard body fat percent cutoffs for diagnosing NWO. There are many gaps in the literature on NWO unlike for overt obesity and future studies should explore the etiology, molecular mechanisms, and adipose tissue changes of NWO as well as conduct well planned and executed randomized controlled trials testing dietary, physical, and behavioral interventions for NWO in both males and females of different racial and age groups.