Melanonychia including melanonychia striata in children poses a diagnostic dilemma. Atypical clinical features often raise the possibility of malignancy, and a nail unit biopsy may be recommended. Commensurate with atypical clinical features, the histopathology may also appear alarming. However, accumulating data illustrate that most cases of melanonychia striata are benign and suggest that an alternate approach is often warranted for pediatric patients. Herein, we review the existing data regarding pediatric melanonychia striata and offer an evidence-based approach to its evaluation and management.