Primary neuroendocrine carcinoma of the skin is a relatively rare tumor that was first described by Cyril Toker in 1972. Since the seminal paper by Toker based on simple morphologic observations and detailed clinical correlation, our understanding of the clinical, morphological, and biological attributes of these lesions has grown exponentially with their increased awareness by pathologists and clinicians as well as with the many contributions of modern diagnostic techniques. The present review focuses principally on the various morphologic appearance that these tumors are able to adopt, the role of modern special techniques for diagnosis, and the conditions that need to be considered in their differential diagnosis.