Pigmentation of the nail plate, or melanonychia, is typically a benign condition caused by melanocyte activation. Although rare, melanonychia may be the initial presentation of melanoma, thus all cases require an in-depth examination. Evaluation in pediatric patients can prove especially difficult as benign cases have a higher prevalence of atypia compared to adults. Lack of specific treatment guidelines in the pediatric population can make diagnosis and treatment challenging. We report a pediatric patient with melanonychia with atypical features that required significant evaluations and collaboration to ultimately reach a treatment plan.