OBJECTIVE: To describe the features of phacomatosis pigmentovascularis 
(cesioflammea type).
DESIGN: Noninterventional retrospective case series composed of 7 patients.
RESULTS: Nevus flammeus combined with ipsilateral ocular melanocytosis or 
melanosis was seen in all 7 patients. Additional contralateral nevus flammeus 
was observed in 3 patients. Nevus flammeus (unilateral in 4 patients and 
bilateral in 3 patients) was distributed in trigeminal nerves V1 (n = 3), V2 (n 
= 7), and V3 (n = 5). Related findings included diffuse choroidal hemangioma (n 
= 1) and glaucoma (n = 1), with no patients having brain hemangioma or seizures. 
Ocular pigmentary abnormalities (unilateral in all 7 patients) included 
congenital ocular melanocytosis (n = 6) and conjunctival acquired melanosis (n = 
1). Pigmentation was sectorial (partial) in 5 patients and complete in 2 
patients. Melanocytosis involved the periocular skin in 1 patient, sclera in 2 
patients, iris in 2 patients, and choroid in 4 patients. In 3 of 6 patients, 
melanocytosis was visible in the choroid only on dilated fundus evaluation. 
Related tumors included choroidal melanoma (n = 3), optic disc melanocytoma (n = 
1), and conjunctival melanoma in situ (primary acquired melanosis) (n = 1). 
Melanoma metastasis developed in 1 patient.
CONCLUSIONS: Phacomatosis pigmentovascularis shows features of nevus flammeus 
and more serious ocular pigmentary abnormalities (uveoscleral melanocytosis and 
conjunctival melanosis). Melanocytosis may be detected only by dilated ocular 
fundus examination, as found in 3 of 6 patients. Furthermore, choroidal melanoma 
can develop from melanocytosis, as noted in 3 of our 6 patients (50%). All 
patients with nevus flammeus should be examined for phacomatosis 
pigmentovascularis by an ophthalmologist because ocular melanocytosis and uveal 
melanoma may remain hidden within the eye.