Filamin a (FLNa) is the first non-muscle actin filament cross-linking protein. It has over 90 binding partners including channels, receptors, intracellular signaling molecules and even transcription factors, which play a role in conformity cell mechanics and signal transduction. Due to this diversity, mutations in human FLNa genes can result in a wide range of anomalies with moderate to lethal consequences including malignant tumor. This review focuses on the research progress of molecule structure and functions of FLNa in diseases development.