Rhizoma Atractylodis macrocephalae is commonly used in Traditional Chinese Medicine. Its traditional functions include treating hypofunction of the spleen with the loss of appetite, abdominal distension diarrhea, phlegm drink dizziness palpitation, edema, fetal movement restless. A literature search was conducted by systematic searching multiple electronic databases including Web of Science, PubMed, CNKI and Google Scholar. Chemical composition analysis of RAM showed that the main compositions were volatile oil, lactones, polysaccharides, amino acids, vitamins and resins. Pharmacological studies indicated that RAM possessed antitumor activities, neuroprotective effect, anti-hepatotoxicity, immune and anti-inflammatory activity, etc. This review gives a detailed description of the chemical constituents, pharmacology and pharmacokinetics of RAM and provides reliable basis for clinical development and application.