A connective tissue nevus is a skin lesion which may be present at birth or appear within the first few years of life.

It is elevated, soft to firm in consistency, varying in size from 0.5 to several centimeters in diameter, and may manifest as grouped, linear, or irregularly-distributed lesions.

[2]: 993 Connective tissue nevi are thickened regions of skin that look as a series of skin-colored papules and plaques.

[3] The majority of reports are of random lesions, but there are some familial cases, indicating autosomal dominant transmission.

[6][7] In addition to hematoxylin-eosin staining to evaluate histological changes, lesion characteristics, genetic history, and special histochemical staining to highlight the composition of connective tissue nevus for differentiation, these tests are necessary for the diagnosis of connective tissue nevus.