The authors report the case of congenital atlantoaxial dislocation in a 9-month-old female infant, who presented with progressive quadriparesis and respiratory failure. The problems in management due to such an early age of presentation, including atypical clinical presentation, unique radiological findings, limited management options, and variable clinical outcomes discussed. This is the youngest case of non-syndromic congenital atlantoaxial dislocation, reported to date, and is a unique combination of lateral, rotatory, and antero-posterior atlantoaxial dislocation. [ABSTRACT FROM AUTHOR]