We report on the combination of biopsy-proven idiopathic livedo reticularis and mononeuropathy multiplex, mild to moderate in severity and remitting-relapsing in course, observed in a young otherwise healthy woman. Neurologic relapses were always accompanied or preceded by exacerbation of the skin lesion. After 18 years followup we did not detect clinical or instrumental evidence of brain or visceral involvement. This excludes the classical Sneddon's syndrome and points at an unusual variant of this syndrome in our patient.