Osteopetrosis is a heterogeneous disorder characterized by abnormal bone remodeling and increased bone density primarily due to defective osteoclast resorption. The diagnosis is based on a history of numerous fractures and radiological findings indicative of osteosclerosis, usually sufficient for a definitive diagnosis. We present a quite rare case of osteopetrosis complicated by recurrent episodes of maxillomandibular osteomyelitis associated with cutaneous fistulization and purulent nasal discharge. We used intravenous antibiotic therapy and necrotic bone debridement that prevented the appearance of acute infections over a 3-year follow-up, but the complete healing of the case was not achieved.