Acute osteomyelitis in neonates, although rare, represents a diagnostic and therapeutic challenge. We herein report an extremely rare case of congenital nonsyphilitic iliac bone osteomyelitis caused by methicilline-sensitive Staphylococcus aureus and presenting with gluteal syndrome in a near-term infant. To our knowledge, this represents the second report with iliac osteomyelitis and the first report with gluteal syndrome in neonatal period. A high index of suspicion is necessary to make an early diagnosis. Early diagnosis is essential to prevent catastrophic sequelae in such cases. We report this case because of its unusual clinical presentation and rarity.