Chest pain is among the leading complaints in paediatric emergency. The Kounis syndrome is a rare allergic coronary vasospasm, which can cause angina and myocardial infarction. Even though organic causes are infrequent as a cause of chest pain in children, it is important to know when and how this symptom may become serious. We reported a patient who had been admitted with chest pain to the emergency department, hospitalised for coronary vasospasm and diagnosed to have Kounis syndrome and heterozygous E148Q mutation. The finding could be a coincidental finding, but considering of the heterogeneity and phenotypic differences in FMF, the relationship between E148Q mutation and Kounis syndrome should be explored further.
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