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Background Although highly effective modulator therapy may reduce future gastrostomy tube (g-tube) placements, a better understanding of the benefits of g-tube placement is needed.Objectives To describe the characteristics of children with cystic fibrosis (CF) who underwent g-tube placement and determine if earlier placement is associated with improved clinical outcomes.Design Retrospective cohort analysis of children with CF born 2003–2019 and followed in the US CF Foundation Patient Registry.Results There were 2337 children with CF (22.9% of the study cohort) who underwent g-tube placement. Compared with children with CF who did not receive a g-tube, a higher proportion of g-tube recipients were diagnosed via meconium ileus or failure to thrive, born at <37 weeks’ gestation or had only class I–III CF transmembrane conductance regulator mutations. G-tube recipients at age 0–1 years who were malnourished had improved body mass index (BMI) z-scores post-placement, though moderately to severely malnourished recipients did not achieve BMI z-scores >0. In contrast, only g-tube recipients at age ≥2 years who were severely malnourished achieved BMI z-scores >0. For g-tube recipients at age 0–1 years, per cent predicted of forced expiratory volume in 1 s (ppFEV1) at age 6–7 years was inversely correlated with malnutrition severity. In contrast, g-tube recipients at age ≥2 years had similar median ppFEV1 at age 6–7 years regardless of malnutrition severity, except those with severe malnutrition had slightly lower median ppFEV1.Conclusions Timing of g-tube placement in children with CF, both in relation to age and severity of malnutrition, was associated with varying degrees of improvements in BMI z-scores and ppFEV1.