Background: The treatment of choice for pilomatricomas is surgical excision; however, data for the optimal timing of treatment and cosmetic outcomes are limited. Objective: This study aimed to investigate the optimal timing of treatment in pilomatricomas by considering clinicopathological findings and cosmetic outcomes. Methods: Seventy-three pilomatricomas patients aged ≤15 years were retrospectively reviewed. Patients were classified into early excision (disease duration ≤12 months, group A) and delayed excision groups (disease duration >12 months, group B). Tumor characteristics, and histopathological features with evolutionary stages were assessed. Cosmetic outcomes were evaluated by the Modified Vancouver Scar Scale (MVSS), 5-point patient satisfaction score, and complication rates. Results: Group A showed better cosmetic outcomes than group B in the MVSS (1.53±1.22 vs. 3.68±1.84), 5-point patient satisfaction score (4.08±0.89 vs. 3.18±1.01), and complication rates (11.8% vs. 36.4%), respectively (p<0.05). Secondary anetoderma, tent sign, calcification, and late regressive stage (evolutionary stage IV) were more common in group B, (p<0.05). Moreover, evolutionary stages showed a positive correlation with mean MVSS (r=0.670, p<0.05). Conclusion: Early excision (disease duration ≤12 months) provides superior cosmetic outcomes compared to delayed procedures. Early recognition, diagnosis, and management for pediatric pilomatricomas is important to improve overall cosmetic outcomes. (Ann Dermatol 32(2) 93∼100, 2020