The co-occurrence of gastroschisis and septo-optic dysplasia, two rare congenital anomalies of unknown etiology, has been reported in four cases. We report a fifth case, and the second where the gastroschisis was on the left side. The case involved a term infant with left-sided gastroschisis who was found to have optic nerve hyperplasia, diabetes insipidus, and hypothyroidism during her initial hospitalization. Gastroschisis and septo-optic dysplasia share some common risk factors, such as young maternal age, smoking, and illicit drug use. Providers should be aware of the potential for hypopituitarism and potential neuro developmental complications associated with gastroschisis, especially in the rare case where the gastroschisis is on the left side.