Primary subungual squamous cell carcinoma (SCC) of the toe is rare in the literature and frequently late or misdiagnosed. Lack of awareness among physicians of the specific clinical findings, the indolent course of the disease, and the high prevalence of benign conditions of the nail unit may account for delays in diagnosis. In addition to difficulties in identifying the true incidence of this tumor, a standardized therapeutic approach does not currently exist. We present a case of subungual SCC of the first toe without bone invasion. The tumor was diagnosed early, treated with conservative surgical excision, and showed no recurrence or metastasis after 2 years of follow-up.