Phakomatosis pigmentovascularis (PPV) can be classified by Classic Hasegawa’s 5 types or new Happle’s 4 types. Though the new Happle’s has some points, it has demerits considering it excluded type I of Classic Hasegawa’s and didn’t cover combined extracutaneous disorders. Classic Hasegawa’s is divided into two subtypes, subtype A and B, based on whether there are only skin manifestations or combined extracutaneous disorders. Although both classifications encompass various congenital skin disorders from keratinocytic, melanocytic, or vascular origin and some extracutaneous disorders, some cases of PPV can’t be classified properly. A 17-month-old female infant presented with brownish macules on left neck, hyperkeratotic brownish plaques on left arm, whitish plaque on right hand dorsum, and erythematous linear patch on both legs. Skin biopsy from each lesion was consistent with agminated lentigines on left neck, epidermal nevus on left arm, connective tissue nevus on right hand dorsum and nevus flammeus on both legs. Discrepancy in the length of extremities was also noted. Therefore, this case was not properly classified by by Classic Hasegawa’s 5 types or new Happle’s 4 types, and so we finally diagnosed her condition as unclassifiable PPV. To the best of our knowledge, there has been no report of PPV with agminated lentigines, epidermal nevus, and connective tissue nevus like this case.