A 15-year-old Japanese girl had widespread annular serpiginous erythematous plaques, bilateral granulomatous uveitis, bloody diarrhea, and seronegative arthralgia. She also had anemia and leukopenia. The histopathologic findings were compatible with those of annular elastolytic giant cell granuloma. Elastolytic granulomas were also found in the cervical lymph nodes, terminal ileum, parietal peritoneum, and mesentery. Bilateral hilar lymphadenopathy, hypercalcemia, and an increased level of angiotensin converting enzyme were not observed throughout the clinical course. To the best of our knowledge, systemic elastolytic granulomatosis has not been previously described in annular elastolytic giant cell granuloma or sarcoidosis. This case may represent a type of granulomatosis in the broad spectrum of annular elastolytic giant cell granuloma and sarcoidosis.