The present paper reports on the case of a 44 year-old woman with progressive facial hemiatrophy associated with bilateral papillitis who developed an acquired complete third nerve palsy on the unaffected side six months later. This Romberg's disease occurred in the context of an immunologic disturbance which was barely improved by steroids and Disulone. Ophthalmic involvement, particularly neurologic involvement, pathogenic hypothesis are reviewed. The responsibility of sympathetic disorder in Parry-Romberg disease is based on frequent association of progressive facial hemiatrophy and Fuch's and Horner's syndromes.