ABSTRACT Cystic lesions of the adrenal gland are rare and difficult to diagnose. Neuroglial heterotopic cysts in the adrenal gland are extremely rare, with few documented cases worldwide. We present the case of a 16-month-old female with a large cystic mass arising from the right adrenal gland. Imaging revealed a sizable cystic lesion, which was resected after converting from robot-assisted to open surgery due to its proximity to major vessels. Histopathology confirmed a mature neuroepithelial cyst, a rare form of neuroglial heterotopia. This case highlights the need to consider neuroglial heterotopia in the differential diagnosis of adrenal cysts in infants and describes our approach to managing this rare lesion.