Introduction:

Ebstein's anomaly is a rare malformation with mild to severe respiratory symptomatic presentation due to congenital heart defects manifested during childhood.

Objective:

To present the case in which the timely diagnosis of this disease allowed the comprehensive treatment of the patient.

Case presentation:

Full-term newborn, who at seven hours of birth presented decreased oxygen saturation, physical examination without cyanosis, pansystolic murmur in auscultation and cardiomegaly detected by chest X-rays. An echocardiogram was performed to diagnose the baby as a carrier of Ebstein's disease with mild manifestations. Once he had clinical improvement, he was discharged with an indication for assessment by pediatric cardiology.

Conclusions:

Ebstein's anomaly may begin in the neonatal period with symptoms of variable severity. Clinical suspicion allows for diagnostic confirmation and close follow-up, especially in severe cases.

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