Leucine-rich repeat containing 8A (LRRC8A) is a member of the LRRC8 family, exhibiting broad expression across various tissues and cells in vertebrates. Like other LRRC8 family members, LRRC8A contributes to the formation of volume-regulated anion channels (VRACs), which are crucial for regulating cell volume and maintaining homeostasis. LRRC8A participates in diverse signaling pathways. Multiple studies have validated the links between LRRC8A dysregulation and neurological disorders, metabolic ailments, and tumors. This review provides a comprehensive overview of the regulatory mechanisms of LRRC8A in these pathologies. The primary goal was to assess the potential of LRRC8A as a therapeutic target for treating diseases and address key unresolved issues.