AIMS: Limited studies have been conducted on juvenile conjunctival nevus (JCN) in Asian populations. This study aims to investigate the clinical and pathologic characteristics of JCN cases among the Han ethnicity in northwest China, providing insights for pathologists and ophthalmologists in diagnosing this condition. METHODS: A subset of conjunctival nevi in children and adolescents, characterized by a confluent growth pattern and lack of maturation, was identified and defined as JCN. A retrospective analysis of the clinicopathologic features of these cases was performed. RESULTS: The study included 40 patients aged 3-20 years, with a median age of 15.5 years. Six cases involved congenital conjunctival pigmentation. Twenty-four were male patients and 16 were female patients, with a male-to-female ratio of 1.5:1. Microscopically, nevus cells grew confluently within or beneath the epidermis and exhibited moderate cellular atypia. In some cases, the junction of the epithelium and the lamina propria layer was obscured by the integration of nevus cell clusters, mimicking epithelial invasion and potentially leading to a misdiagnosis. Over an average follow-up of 78.5 months, no recurrence or postoperative complications were observed. CONCLUSIONS: Diagnosing JCN is challenging because of its histologic resemblance to conjunctival melanoma. A systematic analysis of JCN can improve the recognition of its benign nature by pathologists and ophthalmologists, helping to prevent overdiagnosis of conjunctival malignant melanoma in this young population.