(1) Background: Necrotizing fasciitis (NF) is a severe and aggressive pathology with a rapid progression and high mortality risk. Periocular NF is a rare condition associated with a lower mortality risk but significantly higher disabling sequelae. (2) Methods: We present the case of a 67-year-old homeless patient, a victim of assault, with multiple untreated comorbidities (diabetes mellitus, cardiac conditions, and schizophrenia) and a delayed diagnosis of periocular necrotizing fasciitis. The condition showed a cyclical evolution influenced by the existing comorbidities, and the patient underwent both surgical and medical treatment with a multidisciplinary team. Additionally, we report a systematic review of cases from the literature. (3) Results: The patient's survival outcomes were favorable; however, the sequelae were disabling, not only concerning aesthetic aspects but also due to the loss of the affected eye globe. The systematic review revealed the rarity of such cases and the peculiarities of the presented case compared to those reported in the literature up to this point. (4) Conclusions: Understanding the signs, symptoms, and predisposing factors, as well as the potential rare localizations of NF, including the periocular region, can lead to the early diagnosis and treatment with good functional and aesthetic outcomes, minimizing significant disabilities.
Keywords: enucleation; fascial flap; necrosis; periorbital necrotizing fasciitis; skin graft.