Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome is a recently described chronic inflammatory syndrome consisting of widespread annular violaceous skin lesions and multisystemic inflammatory manifestations. We report a 12½-year-old boy with a young-age onset of recurrent fevers, annular violaceous plaques, alopecia areata, lipodystrophy, low weight and height, deformed fingers, wide-spaced nipples, chronic anemia, and elevated acute phase reactants. An abdominal punch biopsy demonstrated dense perivascular and interstitial infiltrates in the dermis, composed mainly of mononuclear cells. This syndrome may represent a new autosomal recessive auto-inflammatory genodermatosis. Increased awareness may lead to the discovery of more cases, and clarify its pathogenesis.