A 63-year-old woman presented with acute-onset severe dysphagia, for both solids and liquids, with inability to swallow saliva, progressing over the course of 1 week, and ultimately requiring nasogastric tube (NGT) insertion. The dysphagia was accompanied by worsening hoarseness of voice, and it was not associated with odynophagia, stridor, or throat or neck pain. Three days earlier, she developed left pinna pain, which progressed to swelling and erythema, sparing the lobule, and did not respond to amoxicillin. She had also noted mild blurring of vision without eye pain or redness. She did not complain of hearing loss, vertigo, headache, photophobia or other neurologic symptoms. There were no associated constitutional or joint symptoms. No preceding triggers were identified. She had long-standing limited cutaneous SSc, with positive antinuclear antibody (ANA) and negative extractable nuclear antigens (ENAs) with no specific SSc-hallmark antibodies, presenting with Raynaud’s phenomenon for 10 years, sclerodactyly and puffy fingers for 7 years, gastric acid reflux and history of polyarthralgia. Annual screening with echocardiogram and lung function has been reassuring. Her current medication was hydroxychloroquine 200 mg twice daily and losartan 25 mg once daily. Examination revealed an erythematous, swollen left pinna, sparing the lobule (), with no tragus or mastoid tenderness. Neurologic examination revealed a right-sided palatal palsy, but was otherwise unremarkable. Laboratory investigations showed raised C-reactive protein of 21 mg/L (range, 0–5), which was previously normal, and normal renal and liver function with normal urine analysis. ANA was positive (titre 1:1280), with negative ENAs and ds-DNA antibody. Cyclic citrullinated peptide antibody (CCP Ab), rheumatoid factor and anti-neutrophil cytoplasmic antibodies (ANCA) were also negative. Complements C3 and C4 were normal. HIV, hepatitis B virus (HBV), hepatitis C virus (HCV), Influenza A and B, respiratory syncytial virus (RSV) and SARS-CoV-2 were negative. Fibreoptic nasendoscopy showed left vocal cord (VC) palsy. Computed tomography (CT), axial and coronal, scan of the head showed thickening of the left pinna (). Magnetic resonance imaging (MRI) of the head and neck () revealed bilateral thickening enhancement of the glossopharyngeal and vagus nerve complexes, just below the jugular foramen (, arrows), as well as signs of left VC palsy, with enlargement of the left laryngeal ventricle and pyriform fossa, thickening of the left aryepiglottic fold and antero-medialisation of the left arytenoid cartilage (). Intracranial pathology was excluded. CT of neck-chest-abdomen-pelvis excluded malignancy and laryngotracheal compression. Transthoracic echocardiogram was unremarkable, with no evidence of valvulochondritis. The diagnosis of RPC was made clinically supported by the imaging findings. Although this case did not completely fulfil the diagnostic criteria for RPC, the rapid progression with vasculitic involvement of the ninth and tenth cranial nerves, presenting with auricular chondritis, severe dysphagia and voice hoarseness, necessitated immediate treatment. Patient received pulses of intravenous (IV) methylprednisolone 500 mg daily for 3 days, followed by IV hydrocortisone 40 mg four times a day, later switched to oral prednisolone 40 mg daily. Two days after initiating treatment, patient noticed improvement of the pinna erythema, with full resolution in a few days. Blurring of vision also completely resolved in a few days. The dysphagia improved gradually, and NGT was removed on day 15 with the patient being able to swallow liquid and solids, including tablets. Given the rapid progression with CNS involvement, IV cyclophosphamide 12.5 mg/kg/pulse (dose adjusted according to age) was initiated with steroid tapering.