Introduction: Lipomas are benign subcutaneous mass, but the intra-orbital location is rarely reported in the literature and it can resemble a variety of other orbital lesions.
Case report: We describe a 15-year-old girl who presented with left exophthalmia. Orbital magnetic resonance imaging showed an encapsulated intra-conal mass displacing the optic nerve medially, the external right muscle laterally and the globe anteriorly. Excisional biopsy of the mass by lateral orbitotomy approach resolved the exophthalmia, and histology revealed a primary orbital lipoma.
Conclusion: The diagnosis of an intra-orbital lipoma is not easy and the surgical approach represents a challenge to achieve a total excision while avoiding complications.