Gold nanoparticles (AuNPs) play a crucial role in the development of nanomedicine, principally due to their unique photophysical properties and high biocompatibility. The possibility to tune and customize the localized surface plasmon resonance (LSPR) toward near-infrared region by modulating the AuNP shape is one of the reasons for the huge widespread use of AuNPs. The controlled synthesis of no-symmetrical nanoparticles, named anisotropic, is an exciting goal achieved by the scientific community which explains the exponential increase of the number of publications related to the synthesis and use of such type of AuNPs. Even with such steps forward and the AuNP translation in clinic being done, some key issues are still remain and they are related to a reliable and scalable production, a full characterization, and to the development of nanotoxicology studies on the long run. In this review we highlight the very recent advances on the synthesis of the main classes of anisotropic AuNPs (nanorods, nanourchins and nanocages) and their use in the biomedical fields, in terms of diagnosis and therapeutics.