BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) results in progressive replacement of right ventricular myocardium with fibrofatty tissue. This condition commonly presents with sudden cardiac death, cardiac arrest, or sustained ventricular tachycardia among young adults. Considering the likelihood of sudden incapacitation, a diagnosis of ARVC usually signals the end of a career in aviation. However, the morbidity pattern among those diagnosed later in life may differ as compared to the young.CASE REPORT: A private pilot, in his 60s, applied for renewal of an ICAO Class 2 medical certificate a decade after his previous medical certificate had expired. He declared history of hospitalization due to a heart-related condition. Further information revealed that he had been diagnosed as a case of ARVC and that he had declined to be implanted with an implantable cardioverter-defibrillator. Recent clinical examination and related cardiovascular investigations were normal except echocardiogram findings of left ventricular hypertrophy and left atrial enlargement. The treating cardiologist, in the absence of history of syncope and evidence of right ventricular dysfunction or sustained ventricular tachycardia, considered this condition to be stable with minimal risk of malignant arrhythmia.DISCUSSION: This case report discusses aeromedical decision making in a pilot found to have ARVC late in life. Despite the progressive nature of the condition with its attendant risk of incapacitation, considering the age at diagnosis and absence of any high-risk features of ARVC, the regulator decided to issue the medical certificate with safety pilot restriction to enable this pilot to return to aviation activities.Sharma S. Arrhythmogenic right ventricular cardiomyopathy in an older private pilot. Aerosp Med Hum Perform. 2022; 93(2):111–115.