Background: Phakomatosis pigmentovascularis (PPV) is a rare congenital neurocutaneous disorder defined by the coexistence of pigmentary lesions and vascular malformations. PPV type II (phakomatosis cesioflammea) is the most common subtype and is characterized by dermal melanocytosis and capillary malformations. Early recognition is important due to potential ocular, neurologic, and systemic involvement.

Case Presentation: We report a newborn female presenting with extensive slate-gray to blue dermal melanocytosis involving the back, extremities, and abdomen, along with bilateral facial violaceous capillary malformations. Systemic evaluation, including abdominal ultrasonography and brain MRI with angiography, revealed no visceral or intracranial vascular anomalies. Laboratory studies were unremarkable aside from hemoglobin S trait. The cutaneous findings were consistent with PPV type II. Pediatric neurology, dermatology, and ophthalmology referrals were arranged to evaluate for associated glaucoma, neurologic abnormalities, and to initiate long-term surveillance. Future pulsed-dye and Nd:YAG laser therapy was recommended for lesion management.

Discussion: PPV is extremely rare, with fewer than 200 cases documented. The condition may phenotypically overlap with Sturge–Weber syndrome, Klippel–Trenaunay syndrome, and other vascular anomalies, necessitating careful systemic evaluation. Although the pathogenesis is linked to somatic mosaic mutations affecting RAS/MAPK signaling, clinical diagnosis remains paramount. Early multidisciplinary management is essential to monitor for ocular and neurologic complications and to optimize cosmetic outcomes.

Conclusion: This case highlights the characteristic presentation of PPV type II and reinforces the importance of comprehensive systemic evaluation and multidisciplinary follow-up. Awareness of this rare entity aids in distinguishing it from other neurocutaneous syndromes with overlapping features.