Lysophosphatidic acid (LPA), a growth factor-like lysophospholipid, induces diverse cellular responses. The identification of the first LPA receptor gene, through studies of neuroproliferative regions within the embryonic cerebral cortex, has led to the classification of a family of at least eight lysophospholipid receptors with diverse roles in organismal development and function. A growing body of literature has identified roles for LPA signaling under physiological and pathological conditions, particularly within the developing nervous system. Here the authors review features of the LPA receptor family and cellular responses of nervous system-derived cells, and discuss developmental and pathological roles for LPA signaling in the nervous system.