Oral Carcinoma Cuniculatum (OCC), an uncharted variant of oral squamous cell carcinoma (OSCC) is a slow growing tumor with varied clinical presentation, distinctive histopathological characteristics and generally favorable prognosis.

This systematic review was conducted as per the PRISMA guidelines. A comprehensive search was performed on PubMed and ScienceDirect database to extract cases of OCC reported until December 2023. A total of 68 cases were reviewed and the impact of different clinical parameters on the prognosis of OCC as well as common pitfalls in its diagnosis were identified.

R software (version 4.4.2) was used to analyze the data. Cox regression analysis was carried out to assess the impact of clinical variables on survival outcome. Hazard ratio (HR) and 95 % confidence interval (CI) were calculated. Mean age at presentation was 58 years with a male predominance. Mandibular region was the most frequent site of occurrence. Enlargement of lymph nodes, tumor recurrence and metastasis were rare. Surgical management was the primary treatment modality. No significant association between clinical variables and survival was observed.

The unique clinicopathological characteristics and favorable prognosis of OCC distinguish it as a distinct clinical entity. Its indolent growth, non-aggressive clinical features, insufficient tissue sampling and limited awareness amongst clinicians are constant reasons contributing to delayed diagnosis and treatment.