Abstract
BACKGROUND: Anterior sacral meningoceles (ASMs) are rare spinal lesions that may be associated with Currarino syndrome, a condition defined by the triad of anorectal malformations, sacral anomalies, and a presacral mass. While Currarino syndrome and caudal duplication syndrome are distinct entities, their coexistence is exceptionally rare, with only 2 reported cases-none involving an ASM. The authors present a unique case of an ASM in a patient with Currarino syndrome and features of caudal duplication anomaly. OBSERVATIONS: The patient presented with chronic low back pain, paresthesia in the lateral right thigh, and worsening renal function due to hydronephrosis of her solitary left kidney. Imaging revealed a large ASM compressing the left ureter, resulting in severe hydronephrosis and elevated creatinine levels. Surgical intervention included sacral laminectomy, spinal cord untethering, sectioning of the filum terminale, and disconnection of the fistulous connection between the spinal cord and the sacral meningocele. Postoperatively, her renal function improved, and her headache, paresthesia, and back pain significantly resolved. LESSONS: This case underscores the rare coexistence of Currarino syndrome and caudal duplication anomaly, providing valuable insights into the management of an ASM in this setting. https://thejns.org/doi/10.3171/CASE2572.