Ocular surface squamous neoplasia (OSSN) is a spectrum of intraepithelial and invasive neoplastic lesions of the conjunctiva and cornea. OSSN is a rare but potentially sight-threatening ocular malignancy that can be challenging to diagnose due to its clinical and histopathological resemblance to benign ocular surface lesions. However, OSSN can lead to significant ocular and systemic morbidity, including vision loss and metastasis. Various risk factors have been identified, including ultraviolet radiation exposure, human papillomavirus infection, and immunosuppression. The histopathological analysis of the lesion is of utmost importance in diagnosing and further managing squamous cell carcinoma. The acantholytic variant of squamous cell carcinoma is uncommon. Here, we present the case of a 69-year-old male who presented with an invasive mass of progressive growth on the left eyeball extending into the visual axis. The patient underwent extended enucleation, and a histopathological analysis demonstrated a rare acantholytic variant of squamous cell carcinoma.