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Cellular angiofibroma (CAF) is a rare, site-specific benign tumor of mesenchymal origin, which is exclusive of the genital region except very few reported cases of extragenital CAF. Very recently the first case report of orbital CAF has been published and we are presenting the second documented case of CAF in this unusual location, in a 37-year-old man. Magnetic resonance imaging with T2 weighted image revealed a hyper-intense globular mass in the superomedial extraconal region of the left orbit. Histopathology of the excised mass showed numerous bland spindle cells embedded in wispy collagen, suggesting spindle cell tumor. On immunohistochemistry, the specimen was strongly positive to CD34 but negative to desmin, SMA, S-100, C-kit, ERG, EMG, CD31, and CD63, that further confirmed the diagnosis of CAF. CAF is a rare mesenchymal tumor and to the best of the authors’ knowledge, this would be the second documented case of orbital CAF.

Cellular angiofibroma of orbit- a rare tumor at the rarest location