The fruits from the Arecaceae family, although being rich in bioactive compounds with potential benefits to health, have been underexplored. Studies on their composition, bioactive compounds, and effects of their consumption on health are also scarce. This review presents the composition of macro- and micronutrients, and bioactive compounds of fruits of the Arecaceae family such as bacaba, patawa, juçara, açaí, buriti, buritirana, and butiá. The potential use and reported effects of its consumption on health are also presented. The knowledge of these underutilized fruits is important to encourage production, commercialization, processing, and consumption. It can also stimulate their full use and improve the economy and social condition of the population where these fruits are found. Furthermore, it may help in future research on the composition, health effects, and new product development. Arecaceae fruits presented in this review are currently used as raw materials for producing beverages, candies, jams, popsicles, ice creams, energy drinks, and edible oils. The reported studies show that they are rich in phenolic compounds, carotenoids, anthocyanins, tocopherols, minerals, vitamins, amino acids, and fatty acids. Moreover, the consumption of these compounds has been associated with anti-inflammatory, antiproliferative, antiobesity, and cardioprotective effects. These fruits have potential to be used in food, pharmaceutical, and cosmetic industries. Despite their potential, some of them, such as buritirana and butiá, have been little explored and limited research has been conducted on their composition, biological effects, and applications. Therefore, more detailed investigations on the composition and mechanism of action based on in vitro and/or in vivo studies are needed for fruits from the Arecaceae family.