The clinical presentation of skin appendage tumours for the most part is not as specific and diverse as the histological features of this extremely heterogeneous tumour group. Differential diagnosis of certain appendageal tumours can be extremely subtle, yet greatly influence final treatment outcome. Occasional association of certain entities with inherited tumour syndrome s can be extremely important in terms of hereditary cancer screening. This chapter aims to provide the most important clinical, histological and genetic features for selected, most important cutaneous appendage tumours.