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Importance: Foregut duplication cysts are benign developmental anomalies occurring along the foregut-derived portion of the alimentary tract. Several cases of foregut duplications in the head and neck region have been reported, most without airway symptoms. This case is an antenatally recognized anterior tongue lesion leading to respiratory difficulties at birth that was successfully managed by a coordinated fetal care team., Observations: We describe a 4.16-kg female born at full term whose anterior tongue lesion was noted on routine prenatal ultrasound. An airway management plan was developed by a multidisciplinary fetal care team, and the airway was controlled at the time of cesarean delivery. The lesion was completely excised on the sixth day of life without complications., Conclusions and Relevance: Given the extensive differential diagnosis of cystic head and neck lesions in neonates, imaging is recommended to localize and characterize the lesion because management of these lesions may differ substantially. For cases that are diagnosed antenatally, coordination of a multidisciplinary fetal care team and early discussions can optimize the predelivery workup and provide clear delivery and airway management plans. We recommend complete surgical excision of oral foregut duplication cysts in the perinatal period to prevent complications such as feeding difficulties, infection, and ulceration.