Background: Grisel’s syndrome was initially described by Grisel in 1930 to explain the case of a non-traumatic atalanta-axial subluxation (C1 over C2). Exact aetiopathogenesis is unclear with the patient usually presenting as a painful acquired torticollis. Clinical Description: A 9-year-old male child presented with restriction of neck movements, cough for 2 months and tongue deviation for a week. The patient had increased deep tendon reflexes in bilateral upper and lower limbs with bilateral exaggerated plantar reflexes. Power in bilateral lower limbs was 5/5. The patient also had a history of COVID-19 infection 3 weeks back which was managed conservatively. Management: X-ray of the cervical spine revealed subluxation of the atlanto axial joint with an increased atlanto dens interval of 7.5 mm. Magnetic resonance imaging of the Cervical region showed an increased atlanto dens interval of 7.6mm. The patient was immobilised immediately with a hard cervical collar. The patient was started on Inj. Ceftriaxone 850 mg IV twice daily for 21 days, followed by another 21 days of Tab. Cefixime 100 mg twice daily. A repeat X-ray of the cervical spine showed a reduction in atlanto axial dens interval and an improvement in the patient’s condition. Conclusion: Although the diagnosis of Grisel’s syndrome could be challenging at times, it is imperative to manage patients with rare diseases swiftly and precisely to prevent permanent future disabilities. Whether COVID-19 was the trigger for the development of Grisel’s syndrome is up for debate and potential area of new research.