Inflamed conjunctival nevi (ICN) may suggest malignancy because of their rapid growth and atypical histology. The objective of this study was to characterize the diagnostic features of ICN. A retrospective, nonrandomized study of 13 patients with ICN was conducted. A scoring method was developed based on histology and immunopathological parameters. The presence of epithelial solid or cystic inclusions and the preservation of goblet cells are consistent with a benign melanocytic lesion. Periodic acid-Schiff stain and immunohistochemistry to identify the epithelial component prove helpful in the differential diagnosis for melanoma. Polyclonal lymphoid infiltrate and benign cytological features of ICN exclude a diagnosis of lymphoma. Despite the presence of immunoglobulin 4-positive plasma cells in the lesions, ICN does not meet the diagnostic criteria for immunoglobulin 4-related disease. Most patients with ICN are young. The treatment for ICN is complete excision, and the prognosis is excellent.