Primary cardiac neurilemoma, a benign tumor, is extremely uncommon. To our knowledge only eight cases have been reported in the literature. We report a case of a 72-year-old man who presented with complaints of progressive shortness of breath and chest pain, seven years after a right nephrectomy for renal adenocarcinoma. An intra-right atrial tumor was surgically removed; the lesion was found to be a neurilemoma of the right atrium. This case report describes the surgical removal and rarity of neurilemomas, their predisposition to be right-sided in the heart and their coincidental association with other types of cancer.