Introduction: The Authors described a clinical case characterized by an unusual thrombotic event in patient with Systemic Sclerosis.

Clinical findings: We described a clinical case of a younger male with SSc who had a deep vein thrombosis of subclavian vein, axillary vein, humeral vein of the right upper limb in absence of risk factors that could justify this very difficult event.

Diagnosis, therapeutic interventions, outcomes: CT angiography confirmed succlavian vein thrombosis and echo doppler ultrasound study of the right arm which showed thrombosis of the right succlavian vein, thrombosis of axillary vein and thrombosis of humeral vein. The patient underwent therapy with edoxaban, after on month of therapy, he has not yet a complete resolution.

Conclusion: We presented a clinical case of SSc complicated by a venous thrombosis in patient whitout comorbidities treated with NAO (new oral anticoagulant).