Intra-cardiac tumors are rare with estimated prevalence in autopsy series of 0.02%. Atrial myxoma is the most common primary cardiac tumor. The diagnosis can be challenging and high index of suspicion is required for prompt diagnosis and recognition, as symptoms and signs are nonspecific. Atrial myxoma is one of the few emergency diagnoses in cardiac surgery that require urgent consultation and resection to avoid serious embolic complications. We present a case of atrial myxoma with recurrent embolization. We reviewed the literature for prevalence, clinical presentation, diagnostic challenges and treatment of atrial myxoma