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We report a 23 month old gril who presented with right hemiparesis after falling for 6 months. She was also diagnosed with mucopolysaccharidosis type IV (Morquio syndrome) with C1-C2 subluxation. Anesthesia was uneventful after inhalation induction, manual in-line stabilization, intubation, external fixation of the cervical spine, followed by prone position and manual assisted ventilation. Postoperative pain was relieved using a tramadol infusion.