Omphalocele is a congenital malformation of the abdominal wall, which occurs with a frequency of 1-5,000 newborns. The prognosis of treatment often depends on the presence of concomitant malformations. The most common contents of the hernia with omphalocele are the intestinal loops, liver, spleen. However, all organs of the abdominal cavity can be part of the hernial sac with large sizes of omphalocele. Teratoma is a germ cell tumor made up of several different types of tissue, such as hair, muscle, teeth, or bone. They are a type of germ cell tumor and divided into two types: mature and immature. In this article, we describe a rare case of a combination of an omphalocele with a mature teratoma and report the successful single step surgical treatment. On the first day after birth, a simultaneous operation-Removal of teratoma with abdominoplasty was performed. The postoperative period was uneventful, and the child was discharged for recovery.