BACKGROUND Celiac crisis (CC), a potentially life-threatening condition, is one of the rare clinical presentations of celiac disease (CD). Several cases have been documented in the literature, mostly in children. OBJECTIVE To perform a review of CC cases reported in adult CD patients. METHODS A systematic search of the literature was conducted in two databases, PubMed/MEDLINE and EMBASE, using the term "celiac crisis" and its variant "coeliac crisis", from January 1970 onwards. Altogether, 29 articles reporting 42 biopsy-proven cases were found in the search. Here, we summarized the demographic, clinical characteristics, laboratory and diagnostic work-ups, and therapeutic management in these patients. RESULTS Among the 42 CD cases, the median age was 50 years (range 23-83), with a 2:1 female to male ratio. The majority of patients (88.1%) developed CC prior to CD diagnosis, while the remaining were previously diagnosed CD cases reporting low adherence to a gluten-free diet (GFD). Clinically, patients presented with severe diarrhea (all cases), weight loss (about two thirds) and, in particular situations, with neurologic (6 cases) or cardiovascular (1 case) manifestations or bleeding diathesis (4 cases). One in four patients had a precipitating factor that could have triggered the CC (e.g. trauma, surgery, infections). Laboratory workup of patients revealed a severe malabsorptive state with metabolic acidosis, dehydration, hypoalbuminemia and anemia. The evolution of GFD was favorable in all cases except one, in whom death was reported due to refeeding syndrome. CONCLUSIONS Celiac crisis is a rare but severe and potentially fatal clinical feature of CD. A high index of suspicion is needed to recognize this clinical entity and to deliver proper therapy consisting of supportive care and, subsequently, GFD.