Neoplastic lesions (benign or malignant) in the nail region are rare when compared to lesions in the rest of the skin. Despite advances in diagnostic modalities, their diagnosis is frequently delayed or overlooked for days, months, or even years when they are misrecognized or when their approach is not appropriate. Undoubtedly, malignant tumors are the most important lesions since an inopportune diagnosis or treatment can drastically change the patient's prognosis.A review of all the scientific evidence on the two main malignant neoplasms of the nail apparatus (melanoma and squamous cell carcinoma) was carried out using the PubMed search engine from 2003 to 2022, in order to expose the appropriate diagnostic approach and treatment of these nail lesions to avoid delays that obscure the prognosis of patients. This review does not include reconstruction modalities after lesion resection, but the emphasis is placed on the great functional impact they produce. Surgical treatment in the early stages is the most important when talking about prognosis and emphasizing it; systemic oncological management of advanced stages is not so deep.