Nuchal fibromas, uncommon benign soft tissue tumors typically arising along the posterior neck, are often associated with Gardner syndrome (GS). These tumors have rarely been reported in association with scleroderma or as secondary to trauma and diabetes. Nuchal fibromas not associated with GS have been described predominantly in men aged 30 to 50. We report a 2-year-old African American girl with multiple nuchal fibromas along the posterior aspect of her neck and upper back. Retinal examination showed no signs of congenital hypertrophy of the retinal pigment epithelium, and genetic testing for the adenomatous polyposis coli gene mutation seen in GS was negative.