Objective: Very little has been published on longitudinal melanonychia in children. Our objective was to determine the nature of melanocytic lesions in pediatric patients with longitudinal or total melanonychia and to look for correlations between clinical and histologic features. Methods: All patients younger than 16 years of age with longitudinal or total melanonychia who were evaluated at our nail disorder outpatient clinic between September 1993 and September 1996 were included. The clinical and histologic features of the nail condition were determined in each case. Results: Forty patients were included. The final diagnosis was nevus in 19 cases (junctional in 17 cases and compound in 2), lentigo in 12 cases, and functional longitudinal melanonychia in 9. The latter corresponded to a hyperpigmentation caused by melanocytic activation with no increase in the number of melanocytes. None of the patients had melanoma. Appearance within the first year of life, periungual pigmentation, and total melanonychia were consistent features in patients with melanocytic hyperplasia (lentigo or nevus). Early onset of a dark broad lesion in a white patient was typical of melanocytic hyperplasia, although none of these features were pathognomonic. Conclusion: Benign melanocytic hyperplasia (lentigo or nevus) was the cause of 77.5% of cases of longitudinal melanonychia in our overall pediatric population and of 85% of cases in the subset of white patients. All the remaining cases of longitudinal melanonychia were the result of melanocytic activation. (J Am Acad Dermatol 1999;41:17-22.)