Clinically, these lesions can be hard to distinguish from connective tissue nevi such as collagenomas or elastomas. Histologic examination is essential to exclude these entities, which can be associated with congenital abnormalities. The microscopic differential diagnosis is narrow and includes focal cutaneous mucinosis and lichen myxedematosus, both of which have distinct clinical presentations. Lesions of focal cutaneous mucinosis are usually solitary papules and can be found anywhere on the body. Lichen myxedematosus presents with a slow onset of asymptomatic or slightly pruritic papules, which can be generalized or localized. Generalized subtypes of lichen myxedematosus have an associated monoclonal gammopathy, while discrete papular lichen myxedematosus, a variant of the localized subtype, has been associated with human immunodeficiency virus disease.6 Other histologic mimics, like self-healing papular mucinosis and acral persistent papular mucinosis, are easily excluded with clinical information. In our case, clinical correlation confirmed the rare diagnosis of mucinous nevus.