Conjunctival squamous carcinoma, which is regarded as a low-grade malignancy, usually originates at the limbus. Most cases remain superficial to the sclera. Intraocular invasion is rarely reported. We describe a woman misdiagnosed as conjunctivitis and pterygium before a clinical diagnosis of conjunctival squamous cell carcinoma. The diagnosis was made by histopathological examination of the biopsy specimen. Examination revealed an elevated mass on the nasal limbus extending intraocularly. White flaky mass occupied approximately 3/7 space of the anterior chamber. Exenteration was performed for control of local lesion. Histopathologic analysis confirmed that intraocular involvement occurs through the emissary vessels near the area of limbus. The case highlights the need for accurate diagnosis and prompt intervention. A brief review of the clinical and histopathologic features of conjunctival squamous cell carcinoma is also presented. Copyright (c) 2007 S. Karger AG, Basel.