Pseudoexstrophy is a rare variant of the exstrophy/epispadias complex, comprised of the musculoskeletal defects of classic exstrophy but with an intact bladder and urethra. We present a case of a neonate with left-sided gastroschisis and pseudoexstrophy, the first reported combination of these two anomalies. The anomalies in this case were unusual but highlight the importance of careful clinical evaluation before and during surgery so as to carefully define the anatomy of the abnormalities, which will assist in planning surgery and further postoperative management. Identifying and reporting these unusual cases is essential to further expand our understanding of these conditions and their potential associations.