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PURPOSE: To report an elevation deficit, which was caused by an accessory extraocular muscle, in a 6-year-old boy. METHODS: Computed tomography and magnetic resonance imaging were used to confirm an accessory, fusiform, well-defined, solid structure in the retrobulbar space. RESULTS: A supernumerary intraconal muscle was detected between the annulus of Zinn and the posterior part of the left globe. CONCLUSION: This rare anomaly may represent an atavistic retractor bulbi muscle.