Chondrodermatitis nodularis chronica helicis (CNCH) is a benign inflammatory nodule of the helix. Patients report severe tenderness upon pressure. Commonly seen in middle-aged men, there are no reports of this disease in twins. We report middle-aged male monozygotic twins who simultaneously developed CNCH. This suggests, but does not prove, the possibility of a hereditary factor in the pathogenesis of CNCH.