Periodic fever is uncommon in children. The differential diagnosis is large, even though associated symptoms such as rash may help narrow the differential diagnosis. Atypical presentations require thoughtful evaluation. This article describes a case of a 4-year-old boy who presented to the emergency department with recurrent fever, vomiting, abdominal pain, myalgias, and rash. His hospital course is described along with a review on the background, evaluation, management, and complications of tumor necrosis receptor-1 alpha periodic syndrome.