An 11-day-old boy with 22q11.2 deletion syndrome, tetralogy of Fallot, and unilateral renal agenesis presented with a right neck mass and intermittent inspiratory stridor present at birth. The patient did not require positive pressure or intubation, but he had intermittent stridor associated with desaturations. He had feeding difficulty and failure to thrive. On physical examination, the newborn had facial dysmorphism, including a broad and flat nasal dorsum and mild micrognathia. Flexible fiberoptic laryngoscopy revealed a cystic lesion consistent with a vallecular cyst. A modified barium swallow study demonstrated laryngeal penetration and aspiration of thin and nectar-thick liquids. A diffuse, soft, palpable mass was present along the right neck.Magnetic resonance imaging (MRI) demonstrated a multispatial mildly enhancing mass within the bilateral retropharyngeal, parapharyngeal, and carotid spaces, with a mildly hyperintense signal relative to muscle on T1-weighted and T2-weighted images and associated restricted diffusion. The mass was largest on the right side, with convex borders, mild superior displacement of the right parotid gland, and posterior displacement of the right sternocleidomastoid muscle (Figure , A, B). The T2-weighted MRI also demonstrated a hyperintense, nonenhancing lesion within the base of the tongue consistent with a vallecular cyst. The thyroid appeared normal and in its expected location, but no thymus was present in the mediastinum. The patient was taken to the operating room for excision of the vallecular cyst, and a fine-needle aspiration biopsy of the right neck mass was performed for cytopathological analysis (Figure , C, D).