Primary tumors of the heart are presented by different morphological types. One of the rarest forms is a schwannoma of the heart. It consists of the Schwann cells or neurolemmocytes, which produces the myelin constituent of peripheral nerves. Frequency is from 0.02 to 0,056% of all primary tumors. First time it was described in 1972 and it is currently the subject of intense study by clinicians and morphologists, which is associated with the extreme rare diagnostic and peculiarities of the histological structure. Thus far 23 cases of this disease have been reported in the literature, 8 of them located primarily in the right atrium. During the study of this type of tumor in 1920, the Swedish scientist N. Antoni identified two types of tumors depending on the type of cellular architecture (type A and type B). Type A consists of spindle-shaped cells in the form of bundles, and type B consists of polygonal cells with abundant cytoplasm and they are not tightly arranged. Also, in addition to identifying specific cells, the diagnosis of schwannoma can be confirmed due to the strong diffuse staining of the S100 protein. The only method of treatment is surgical correction with the replacement of the tissue deficit with various biological and synthetic materials after tumour resection. If it is impossible to perform a complete excision of the tumor, heart transplant is the only kind of treatment.