Group 2 innate lymphoid cells (ILC2) exert critical roles in type 2 immune responses, epithelial repair at mucosal tissues and metabolic homeostasis. ILC2 rapidly provide large amounts of type 2 signature cytokines, thereby driving type 2 immune responses such as the defense against helminths. However, if deregulated, ILC2 facilitate tissue fibrosis and trigger unwanted type 2 immunopathologies such as allergies, asthma and atopic dermatitis. Therefore, ILC2 need to be tightly regulated and we are just beginning to understand which mediators activate or inhibit this rare but important cell population. In this review, we summarize current knowledge about positive and negative regulation of ILC2 and discuss its immunological consequences.