Cardiac tumors are uncommon conditions in medical practice, and among them, the most prevalent is the myxoma. Despite their benign nature, myxomas may have behavior suggestive of malignancy and serious consequences. They may have cardiovascular repercussions associated with nonspecific systemic manifestations, which often hinder their diagnosis, benefiting their evolution and increasing the risks of serious complications, including embolic alterations and death. We present, in this work, the case report of a child admitted to the Hospital Infantil Público de Palmas (HIPP), with clinical history of abdominal distension, adynamia, hyporexia and intermittent fever, initially diagnosed with right atrial myxoma. The patient had different epidemiological characteristics than those described in the literature for cardiac myxomas. This, combined with the fact that cardiac myxomas have a low incidence and commonly present in non-specific cardiovascular and constitutional manifestations, made it difficult to diagnose and delay surgical treatment. Attention to the possibility of this disease and the echocardiogram in the evaluations may facilitate the early diagnosis of this benign cardiac neoplasia.

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