An eighteen-year old man is reported who had a large nevus spilus on the right side of his body with port wine stain on the anterior and posterior sides of his trunk without any systemic involvement. Considering the clinical signs and skin biopsy report of the patient, the diagnosis of phakomatosis pigmentovascularis type IIIa was made.

We report phakomatosis pigmentovascularis (PPV) detected in a 40- year-old male characterized by the presence of a port-wine stain in the background of aberrant Mongolian spots covering the back, nevus of Ota, ocular melanosis, epidermal nevus and a scaly patch with the diagnosis of discoid lupus erytematosus. These associations have not been reported yet.