The dysplastic melanocytic nevus remains an issue of controversy despite extensive investigations. On clinical grounds the term atypical melanocytic nevus should be used, while dysplastic melanocytic nevus describes histological characteristics. The association with melanoma is complex. With the clinical picture, dermatoscopy and molecular biological or genetic examinations one can often not distinguish a histological dysplastic nevus from a melanoma. In patients with large amounts of melanocytic nevi it is important to assess the total melanoma risk, the need for patient surveillance and motivate the patient for self-examination. In high-risk patients the amount of benign melanocytic nevi is increased and many clinically atypical and microscopically dysplastic nevi can be found. The relatives of these patients should also be examined. Because of the rising incidence of melanoma and the lack of therapeutic options in disseminated disease, the surveillance of high risk patients, the early detection of melanoma and excision play a key role in patient management.