Purpose: To report a case of bilateral malignant external otitis in a patient with severe heart disease. CASE REPORT: A 56-year-old man, retired with severe heart disease in irregular treatment, presented 20 days after developing itching in his right ear. The patient developed a right earache associated with swelling and ipsilateral otorrhea and did not improve with antibiotic therapy. Ectoscopy showed the presence of necrotic tissue in the pinna, external auditory canal, and right helix in the left ear. Computed tomography of the mastoid showed swelling in the right external auditory canal without opacification of the mastoid cells. Gallium-67 scintigraphy showed an area of increased contrast uptake in the right ear. Surgical debridement of both ears was performed, and the patient underwent hospitalization, cardiac monitoring, intravenous ciprofloxacin, and analgesia treatments daily for 3 weeks. The resolution was satisfactory after 8 weeks of treatment, but patient developed stenosis of the right external auditory canal with the same resolution as in the left ear. The patient was discharged after 2 weeks of treatment, and is being followed weekly at the clinic. The patient underwent surgery for mitral valve replacement, progressing unsatisfactorily in the postoperative period. Death occurred 4 days after surgery. CONCLUSION: Malignant external otitis is an aggressive infectious and potentially fatal disease that affects mainly elderly patients, diabetics, and the immunocompromised. The correct treatment and control of systemic diseases are essential to the satisfactory progress of malignant external otitis.