Annular elastolytic giant cell granuloma is a rare disease characterized by annular configurated lesions with typical histopathologic findings. We report a case of annular elastolytic giant cell granuloma in a 50-year-old male patient. Treatment modalities for this disease are limited and mostly unsatisfactory, although isotretinoin was found to be effective in a single case. However, systemic isotretinoin treatment of 12 weeks' duration was of no benefit in the presented case.