A growing number of Campylobacter species other than C. jejuni and C. coli have been recognized as emerging human and animal pathogens. Although C. jejuni continues to be the leading cause of bacterial gastroenteritis in humans worldwide, advances in molecular biology and development of innovative culture methodologies have led to the detection and isolation of a range of under-recognized and nutritionally fastidious Campylobacter spp., including C. concisus, C. upsaliensis and C. ureolyticus. These emerging Campylobacter spp. have been associated with a range of gastrointestinal diseases, particularly gastroenteritis, IBD and periodontitis. In some instances, infection of the gastrointestinal tract by these bacteria can progress to life-threatening extragastrointestinal diseases. Studies have shown that several emerging Campylobacter spp. have the ability to attach to and invade human intestinal epithelial cells and macrophages, damage intestinal barrier integrity, secrete toxins and strategically evade host immune responses. Members of the Campylobacter genus naturally colonize a wide range of hosts (including pets, farm animals and wild animals) and are frequently found in contaminated food products, which indicates that these bacteria are at risk of zoonotic transmission to humans. This Review presents the latest information on the role and clinical importance of emerging Campylobacter spp. in gastrointestinal health and disease.