Background: Keratoacanthoma is a relatively common cutaneous neoplasm traditionally regarded as a low-grade or self-limiting variant of squamous cell carcinoma. Perineural invasion (PNI) is uncommon in keratoacanthoma and is typically associated with more aggressive cutaneous malignancies, carrying important prognostic and management implications. Case Presentation: We present the case of a 47-year-old female who underwent excision of a clinically suspected keratoacanthoma. Histopathological examination demonstrated features consistent with keratoacanthoma with unexpected perineural invasion in the form of two 0.2mm diameter nerves. Surgical margins were clear, however re-excision for wider margins was performed given the high-risk features and lack of access to adjuvant radiation treatment in the patient’s local area. Review: A literature review was performed to identify reported cases of keratoacanthoma associated with perineural invasion. Cases were analysed for patient demographics, tumour location, histological features, management strategies, and clinical outcomes. This literature review identified a small number of reported cases demonstrating perineural invasion in lesions diagnosed as keratoacanthoma. These cases show variability in management, ranging from wide local excision alone to adjuvant radiotherapy, reflecting ongoing uncertainty regarding the biological behaviour and optimal treatment of keratoacanthoma with high-risk features. Conclusion: Perineural invasion in keratoacanthoma is rare but clinically significant. This case highlights the importance of thorough histopathological assessment and multidisciplinary evaluation when high-risk features are identified. Greater awareness and further reporting of similar cases are required to guide risk stratification and inform management recommendations.