Desmoid tumor is a rare locally progressive tumor represented by fibroblasts. Unlike other malignancies, desmoids do not metastasize. However, they can cause serious problems due to local spread. These neoplasms can have different localization, but are most often diagnosed in anterior abdominal wall. Desmoids can pose specific clinical challenges for surgeons and patients. This disease is extremely rare. Thus, there are few randomized studies devoted to effectiveness of each treatment method. This circumstance necessitates further research in these patients to assess the effectiveness of treatment. The authors describe a patient with large desmoid fibroma of anterior abdominal wall. This clinical case represents a unique opportunity to analyze the features of diagnosis and treatment of desmoid tumor. This material will be especially useful for abdominal surgeons, oncologists and morphologists.