Idiopathic pulmonary fibrosis (IPF) is a progressive and irreversible lung disease characterized by fibrosis in the lung parenchyma and collagen deposition leading to respiratory failure. Different etiopathogenetic hypothesis have been formulated during the last years and many studies recently published demonstrated that in most of processes suggested for the onset and the development of IPF, chemokines and chemokine receptors are involved. Dysregulated expression of chemokines and their receptors during inflammatory processes might also alter the equilibrium between angiostatic and angiogenic processes leading to neovascularization in the lung tissue. Studies on chemokines/chemokine receptors could shed light on the mechanisms involved in IPF and draw new therapeutic strategies to block the progression of the disease.