Background: In recent years, there has been a growing international interest in family quality of life The objective of this systematic review is to understand and analyze the conceptualization of the quality of life of families with children with disabilities between 0 and 6 years of age, the instruments for their measurement and the most relevant research results. Method: A bibliographic search was conducted in the Web of Science, Scopus and Eric databases of studies published in English and Spanish from 2000 to July 2019 focused on “family quality of life” or “quality of family life” in the disability field. A total of 63 studies were selected from a total of 1119 and analyzed for their theoretical and applied contributions to the field of early care. Results: The functional conceptualization of family quality of life predominates in this area, and a nascent and enriching holistic conceptualization is appreciated. There are three instruments that measure family quality of life in early care, although none of them is based on unified theory of FQoL; none of them focus exclusively on the age range 0–6 nor do they cover all disabilities. Conclusions: The need to deepen the dynamic interaction of family relationships and to understand the ethical requirement that the methods used to approach family quality of life respect the holistic nature of the research is noted.