ObjectiveThis study aimed to review and summarize the clinical presentation and pathological features of localized juvenile spongiotic gingival hyperplasia (LJSGH) to enhance pediatric dentists’ understanding of this condition.MethodsA retrospective analysis was conducted on the clinical and pathological data of patients with LJSGH diagnosed in the Department of Oral Pathology, Tianjin Stomatological Hospital from January 2019 to December 2024.ResultsTwelve cases were retrieved, the male-to-female ratio was 1.4∶1, and nine of the cases were children and adolescents (<18 years old). the typical clinical presentation was characterized by erythematous, hyperplastic gingiva exhibiting a papillary or granular appearance. the lesion was predominantly solitary and primarily located on the labial gingiva of the maxillary anterior region. the typical histopathological features were as follows: the superficial epithelium of the lesional area consisted of nonkeratinized stratified squamous epithelium with intercellular edema and spongiosis; irregular elongation of rete ridges; neutrophil infiltration in the superficial epithelial layer; marked vascular dilation and congestion in the subepithelial papillary layer; and a distinct boundary between the lesional epithelium and adjacent normal mucosal epithelium. ck19 exhibited strong positive expression throughout the lesion epithelium. by contrast, only the basal layer showed a strong positive expression in normal gingival tissues.ConclusionLJSGH exhibits distinctive clinical features, and its diagnosis can be confirmed through histopathological examination. The fin-dings of this study contribute to enhancing pediatric dentists’ understanding and recognition of LJSGH.