The nail unit can be a challenging anatomic location for surgical removal of neoplasms. Although uncommon, malignancies do affect this specialized epithelial structure. In particular, Bowen's disease and more invasive squamous cell carcinoma (SCC) are the most common neoplasms to affect the nail unit and surrounding structures. Other neoplasms such as basal cell carcinoma and malignant melanoma can also affect the nail unit, but less frequently, and will not be discussed in the scope of this review. Mohs micrographic surgery continues to be the treatment of choice because of the procedure's tissue-sparing qualities. A clear understanding of the anatomy and the histology of the nail unit, a review of the technique of Mohs surgery of the nail anatomy, as well as a review of the literature are presented.