The authors describe four patients with Parry-Romberg syndrome (PRS) who had abnormal eye movements, ptosis, and facial hemiatrophy. There were delays in diagnosis in all cases, often requiring multiple imaging studies, prior to diagnosis of PRS. These cases demonstrate the variable ophthalmic manifestations of PRS, which can lead to difficulty in diagnosis. Case 1 was a 23-year-old woman with lateral rectus paresis who presented with horizontal diplopia and developed ptosis and facial hemiatrophy. Case 2 was a 28-year-old woman with medial rectus weakness adjusted to diplopia by head turn and gaze preference. Case 3 was a 68-year-old woman who presented with dry eye symptoms and was noted to prefer right gaze and have left hemifacial atrophy. Case 4 was a 68-year-old woman who presented with ptosis, enophthalmos, and restriction of up gaze. The ocular motor defect in all of these cases is best explained by muscle fibrosis rather than nerve paresis.
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