Desmoplastic small round cell tumor is a very rare soft tissue sarcoma with a bleak prognosis and short patient survival. The most common occurrence is in 20-30-year-old men. Our study presents the case report of a 40-year-old patient who was diagnosed with this sarcoma. The first symptom of the illness was an incarcerated epigastric hernia with sarcoma metastasis resembling an intestinal loop in an ultrasound image. The fluorescence in situ hybridization (FISH) method showed a fusion of the EWS and WT1 genes. Systemic palliative chemotherapy using the VDC-IE (vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide) regimen was chosen instead of further surgery due to the disease's generalization. However, the therapy failed to halt the disease progression and was thus terminated after 18 months. The patient's overall survival was 19 months. The rare character of this disease complicates the diagnostics in clinical practice. Nevertheless, rare sarcomas should be considered in patients with non-specific abdominal symptoms, including patients with incarcerated ventral hernia.