| Summary: | Purpose: To present the clinical, histological, and radiographic findings of a case of orbital myofibroma in an unusual location. The literature is reviewed and the clinical relevance discussed. Methods: A 5-year-old boy was examined with a 1.5-month history of progressive swelling in the left supraorbital region. Results: Examination revealed a firm, painless mass in the supralateral region of the left orbit with slight reddish discoloration of the overlying skin. Computerized tomography (CT) scan images showed a well demarcated, homogenous, solid mass with extension to the lacrimal gland region and adjacent to frontal bone erosion. The mass was surgically excised and was confirmed to be myofibroma in diagnostic histological studies. There has been no evidence of recurrence in the first year after surgery. Conclusions: Clinical appearance and imaging findings are unspecific for this tumor, and histological examination still remains the definite method of diagnosis. Therefore, it is important to be able to differentiate myofibromas from other malignant tumors with a similar presentation in pediatric patients to avoid mismanagement. Keywords: Orbital tumor, Lacrimal gland, Children, Histopathology
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