Objective:To describe a case of neonatal atrial flutter after the insertion of an intracardiac umbilical venous catheter, reporting the clinical presentation and reviewing the literature on this subject.Case description:A late-preterm newborn, born at 35 weeks of gestational age to a diabetic mother and large for gestational age, with respiratory distress and rule-out sepsis, required an umbilical venous access. After the insertion of the umbilical venous catheter, the patient presented with tachycardia. Chest radiography showed that the catheter was placed in the position that corresponds to the left atrium, and traction was applied. The patient persisted with tachycardia, and an electrocardiogram showed atrial flutter. As the patient was hemodynamically unstable, electric cardioversion was successfully applied.Comments:The association between atrial arrhythmias and misplaced umbilical catheters has been described in the literature, but in this case, it is noteworthy that the patient was an infant born to a diabetic mother, which consists in another risk factor for heart arrhythmias. Isolated atrial flutter is a rare tachyarrhythmia in the neonatal period and its identification is essential to establish early treatment and prevent systemic complications and even death.