Postoperative immunohistochemistry indicated SMA (+), Calponin (+), Ki-67 (+, proliferation index of 20%), Desmin (-), S-100 (-), CD34 (-), CK (-), leading to the final diagnosis of infantile solitary orbital myofibroma.<h4>Conclusion</h4>Infantile orbital myofibroma is clinically rare and frequently misdiagnosed as schwannoma, which requires immunohistochemical and molecular genetic testing for definitive diagnosis. The gene discussed is MKI67; the disease is schwannoma.