A middle childhood boy with no disclosed medical history presented with paroxysmal episodes of pain in the left ear accompanied by redness of the earlobe over a 3-month period. The attacks occurred suddenly, lasted for hours and had a variable frequency. He denied experiencing a headache or other neurological symptoms and had previously attempted treatment with gabapentin, prednisolone, ibuprofen and topical lidocaine without success. Suspecting red ear syndrome (RES), we requested cervical and brain MRI and initiated a therapeutic trial with indomethacin 75 mg/day. The MRI revealed a loop of the right anterior inferior cerebellar artery in the internal auditory canal, without pathological significance. An improvement in both the frequency and intensity of the paroxysms was observed. Subsequently, indomethacin was gradually tapered, and treatment with flunarizine was introduced, yielding a favourable response. RES is a rare disorder with an unknown pathophysiology, and its optimal treatment approach remains largely empirical.