Amyloids are common protein aggregates in nature. Some amyloids fulfill important biological tasks while others are known to cause diseases. Despite the fact that the ultrastructure of amyloid is highly conserved, the mechanism of amyloidogenesis remains a challenging research topic. In humans, amyloidoses may develop in the skin or lead to skin signs due to secondary cutaneous involvement. An accurate diagnostic procedure is crucial for planning the therapy of this heterogeneous group of diseases. Therefore, the aim of this paper is to give an overview on the different kinds of amyloidoses as well as on diagnostic and therapeutic approaches. Furthermore, the discrimination between functional and disease-causing amyloid is briefly presented.