Childhood-onset long-lasting recurrent fevers with severe abdominal pain, myalgia/arthralgia, rash, and a positive family history are indicative of TRAPS, and all patients suspected to have TRAPS should be evaluated by genetic testing for germline variants in TNFRSF1A. Classification criteria that include genotype outperform classification criteria based on clinical features by sensitivity and specificity. Here, TNFRSF1A is linked to TNF receptor 1-associated periodic fever syndrome.