Objective:

To report a case of primary angiomyofibroma of soft tissue (AFST) in the small intestine presenting with intussusception and to review the literature on this rare tumor, highlighting its clinical significance.

Key Findings:

• AFST is a rare benign mesenchymal neoplasm, primarily found in extremities, with no prior reports in the small intestine, underscoring the need for increased clinical awareness.
• The tumor caused intussusception due to luminal obstruction, highlighting the potential for significant complications.
• Histological and immunohistochemical features supported the diagnosis of AFST, reinforcing the importance of accurate diagnosis.

Interpretation:

The case highlights the rarity of AFST in the gastrointestinal tract and the potential for significant complications like intussusception.

Limitations:

• Molecular genetic studies were not performed to confirm the diagnosis, which may limit the understanding of the tumor's characteristics.
• The literature review may not encompass all cases due to the rarity of the tumor, potentially affecting the comprehensiveness of the findings.

Conclusion:

AFST can present in unusual locations such as the small intestine, and complete surgical excision is recommended due to the risk of local recurrence, emphasizing the need for clinician awareness.