Orbital lymphomatoid granulomatosis – a rare cause of proptosis

A 1-year-old girl with unilateral proptosis was found to have primary orbital lymphomatoid granulomatosis – a condition rarely occurring in children. This multisystem angiocentric, angiodestructive, lymphoproliferative disease typically involves the lungs, with ocular involvement being extremely uncommon. Our case serves to illustrate the imaging findings of this unusual condition and highlight a rare cause of proptosis.