Purpose: To evaluate the relationship between nutritional status and surgical outcomes in patients with esophageal atresia (EA) from the Turkish Esophageal Atresia Registry (TEAR). Methods: Between 2015 and 2024, 713 patients with the complete data of neonatal period and first year of life were included. According to FENTON, growth charts and patients were grouped as small for gestational age (SGA, < 10 percentiles), medium SGA (percentiles = 10–20), appropriate for gestational age (AGA, percentiles = 20–90) and large for gestational age (LGA, percentiles > 90) at birth. The z scores for height-for-weight were reevaluated at 6th and 12th months of age. Results: Among 713 patients, 56% were boys. 23.7% of patients were SGA. There was no difference among groups for demographic features, outcomes, and mortality (p > 0.05). Patients with SGA had a higher rate of karyotype anomalies (23.1%, p < 0.05). At the 6th month, 20% of patients had improved nutritional status, 46.2% unchanged, and 33.5% worsened. At the 12th month, it was 31.6%, 50.2%, and 18.3%, respectively. 32.8% of the SGA patients had severe malnutrition at the 6th month, while this rate decreased to 10.2% at the end of the first year of life. Patients with worsened nutritional status had a significantly higher rate of mortality (10.2%) than patients with unchanged and improved nutritional status (3.7%, 2%, respectively, p < 0.05). There was no statistical difference between nutritional status and surgical outcomes at the 6th and 12th months (p > 0.05). Conclusions: The incidence of SGA was significantly higher in EA patients with karyotype anomalies. While 20% of patients improved nutritional status at the 6th month, only one-third of patients improved nutritional status at the end of the first year. Closer follow-up is needed in patients with EA to avoid malnutrition, which can lead to poor growth, developmental delay, and impaired immune function.