PURPOSE: To describe a previously unreported association between phakomatosis pigmentovascularis (PPV) IIa and primary choroidal melanoma. DESIGN: Case series. PARTICIPANTS: Three patients with PPV type IIa and choroidal melanoma. RESULTS: Nevus flammeus was present unilaterally in each patient, involving the hemiface, the hemithorax, or both. Ocular melanocytosis also was present unilaterally in all patients involving the sclera, the iris, the choroid, the distribution of the trigeminal nerve, or a combination thereof. Renal cysts were noted in patient 1, and a nevus anemicus of the neck was noted in patient 3. A unilateral choroidal melanoma was detected in each of these 3 patients, in each patient involving the same eye in which melanocytosis had been seen. No metastases were found in any patient. CONCLUSIONS: Clinicians should be aware of the possibility that PPV, and in particular that occurring in patients with ocular melanocytosis, can be associated with choroidal melanoma.