Neuroendocrine neoplasms develop from diffuse neuroendocrine system cells and occur rare. They characterized by insidious progress and often are recognized accidentally. We present a case of a patient, who had a nephrectomy of the left kidney, due to a papillary carcinoma. While the operation, there had been discovered a focal change of mesenterium, which was taken a biopsy from. On histopathological examination, neuroendocrine neoplasm has been confirmed. The patient was treated with a long-acting somatostatin analogue. During a further observation, progression of the disease has been observed and cardiac metastasis of the neuroendocrine neoplasm was discovered, what is uncommon. Due to progression, peptide receptor radionuclide therapy was added to chronic treatment with the longacting somatostatin analogue. Stabilization of the disease have been achieved. After 1,5 year the second progression has been observed and the peptide receptor radionuclide therapy was used one more time, which was successful. We also describe a diagnostic difficulty, caused by poor sensitivity of the most often used imaging methods of the heart, like echocardiography and magnetic resonance imaging, which are not as effective as a molecular imaging methods to recognize metastases of neuroendocrine neoplasms. Knowledge about cardiac metastases of neuroendocrine neoplasms still is little. Their impact on a course of disease and prognosis still is not known. For this reason, more investigations are needed.