The true bifid epiglottis is a rare congenital anomaly typically disco
vered during the evaluation of strider in an infant or newborn. While
it is not classified as a specific syndrome, there are frequent associ
ations of other congenital anomalies with the bifid epiglottis. These
include midline defects (such as microphallus, hypospadius, imperforat
e anus, and midline laryngeal cleft), endocrine disorders (including c
ongenital hypopituitarism), and central nervous system neoplasms, incl
uding hypothalamic hamartoblastoma. The embryogenesis and options for
surgical management of this anomaly are reviewed, and one case is pres
ented in detail.