Rationale: Pleomorphic rhabdomyosarcoma (PRMS) is a rare soft tissue malignancy which is frequently misdiagnosed and associated with metastasis to the lungs, lymph nodes, and bone marrow. Case studies are needed to improve the awareness of the disease and our understanding of it.
Patient concerns: In this study, we present a case of a 36-year-old man with a lesion on the right back shoulder. Lesion was confirmed by magnetic resonance imaging (MRI) around the right armpit, subscapularis, deltoid, and infraspinatus muscle, with oozing surrounding soft tissues.
Diagnosis: The tumor was diagnosed as PRMS which metastasized to the intestine, where it caused intussusception.
Interventions: The patient was treated by complete surgery in combination with neo-adjuvant chemotherapy including ifosfamide and epirubicin.
Outcome: The patient remained alive 6 months after the treatment with no recurrence and metastasis.
Lesson: PRMS can be aggressive, and surgical treatment in combination with multidrug chemotherapy can be used in the management.