PURPOSE: To clarify the clinical and pathologic findings of 7 patients with inflamed juvenile conjunctival nevus (IJCN) treated with tacrolimus. STUDY DESIGN: Retrospective study. SUBJECTS AND METHODS: The medical records of 7 male patients diagnosed with IJCN between February 2007 and October 2022 at the Kansai Medical University Hospital and Ideta Eye Hospital were retrospectively reviewed. The patients' mean age was 11.3 (8-20) years, and the average follow-up period was 18 months. The patients underwent anterior segment optical coherence tomography (AS-OCT) and anterior segment optical coherence tomography angiography (AS-OCTA). All the patients were treated with steroids or immunosuppressant eye drops, or both. Histopathologic examinations were performed on specimens resected from 2 patients. RESULTS: The clinical characteristics of IJCN were as follows: (1) presence of cysts within a pigmented conjunctival mass, (2) tumor accompanied by feeder vessels, and (3) predominantly found in boys with allergic diseases. AS-OCT and AS-OCTA revealed the presence of a cystic structure with poor vascular signals within it and numerous vessels distributed on the sclera underlying the lesion. Immunosuppressant eye drops relieved the congestion, diminished the inflow vessels, and induced shrinkage of the cysts in all the patients. Histopathologic examinations revealed infiltration of inflammatory cells such as lymphocytes and macrophages and low proliferative activity of melanocytes with no division pattern. CONCLUSIONS: This study clarified the clinical and histologic characteristics of IJCN. Noninvasive AS-OCT and AS-OCTA might be useful for diagnosing IJCN. The histopathologic findings of this study indicate that immunosuppressive and antiallergic agents can effectively bring about disease remission.