Abstract
We report an 11-year-old North Indian girl presenting with a flesh-colored, asymptomatic, slowly enlarging raised lesion with cerebriform architecture on the dorsum of her right hand since birth. Routine investigations were unremarkable. A punch skin biopsy confirmed the diagnosis. Clinical and histopathological findings established the lesion as a fibroblastic connective tissue nevus, a rare developmental anomaly in an uncommon acral location, which posed a diagnostic challenge. This case is particularly novel due to its acral location on the hand.