Introduction: Research on quality of life exclusively in the context of extreme prematurity and preschool is almost non-existent. Objective: The purpose of this descriptive correlational study was to describe the quality of life of children born extremely premature, during infancy, according to maternal perception. The specific objectives were to describe the different dimensions of the quality of life of children born extremely premature and to explore the relationships between certain sociodemographic and clinical variables and quality of life. The study was based on Callista Roy's adaptation model (1976). Method: Forty-two parents of children born extremely premature and aged 2 to 5 years responded to the PedsQL 4.0 during telephone interviews. Results: Overall, the results show that the children have a good quality of life, as perceived by the mothers. Physical functioning was the most optimal dimension of quality of life, while emotional functioning was the least optimal. Furthermore, children from nuclear families have a better global quality of life than children from other family types. Discussion and conclusion: Extreme prematurity requires careful monitoring by nurses to ensure healthy development of toddlers and therefore good quality of life. Future research is needed to further document the quality of life of children born extremely premature at preschool age.