Multiple connective tissue nevi in linear arrangements, which was previously described as zosteriform connective tissue nevus, is a rare variant of connective tissue nevus. We herein report the case of an 8-year-old Japanese boy with this disease. He developed a small mass on the upper region of his right inner ankle at the age of one-half years. Then, other lesions had appeared on the inner side of his right lower leg, thigh, and groin within the next 2 years. Furthermore, multiple new lesions in linear arrangements had appeared on the right half of the abdomen and on the extensor side of the right forearm at the age of 8 years. A biopsy specimen revealed abnormalities in both the collagen bundles and the elastic fibers. Since the distribution was along Blaschko's lines, not dermatomes, we propose that the diagnosis "linear connective tissue nevus" is suitable for this clinical study.