Food neophobia is defined as the unwillingness to taste new foods and the avoidance of unfamiliar foods. This eating behaviour is a complex issue, and both genetic and environmental factors play a role in it. The aim of this review is to understand its relationships with dietary behaviours throughout the life span and to examine the impact of interventions on food neophobia. A literature search was performed using the PubMed, Web of Science, Cochrane Library, and ScienceDirect databases. As a result of the screening, a total of 139 studies, 17 of which were intervention studies, were included in this review. According to current evidence, food neophobia is negatively associated with the acceptance of not only novel/unfamiliar foods but also familiar foods. Many studies have shown that food neophobia is negatively associated with the hedonics and consumption of core foods, especially vegetables and fruits. Individuals with higher levels of food neophobia are less familiar with many foods, but familiarity is a prominent motivator in food choices for these individuals. Therefore, it may be considered a barrier limiting diet quality and this trend is similar for both children and adults. However, food neophobia is not an unchangeable personality trait. Intervention studies have pointed out that educational programs and food-related activities that increase familiarity and exposure to foods and create positive attitudes toward and positive experiences with foods can reduce food neophobia. The results highlight that people with high food neophobia need more support to improve their diets and the quality of dietary behaviour.