Asteraceae is the world's largest family of flowering plants, consisting of 23000 species distributed worldwide in 13 subfamilies, 44 tribes and more than 1600 genera. Achillea L. is one of the youngest evolutionary genera of the family Asteraceae with a worldwide widespread distribution of more than 100 species. Turkey is one of the main diversity centers of the genus Achillea. A total of 47 Achillea species are distributed and naturally growing in Turkey, 24 of which are endemic to Turkey with 51% endemism rate. Achillea species are used as tonic, anti-inflammatory, anti-spasmodic, diaphoretic, diuretic and emmenagogic agents and have traditionally been used to treat bleeding, pneumonia, skin disorders, rheumatic pains. Achillea species have several phytoconstituents such as essential oils, sesquiterpenes and phenolic compounds such as flavonoids and phenolic acids. Flavonoids and phenolic compounds are the most important effective metabolites of Achillea species. Phenolics and flavonoids have been reported to exert a wide range of pharmacological activities, including anti-cancer, anti-inflammatory, and antioxidant effects. In this review, endemic Achillea species were reviewed in terms of phytochemical content and pharmacological activities.