BACKGROUND: Atrophoderma vermiculatum is a rare, benign follicular disorder that primarily affects children. It is characterized by symmetric reticular or honeycomb atrophy of the cheeks that may extend to the ears and forehead. The defect is believed to be due to abnormal keratinization in the pilosebaceous follicle. Management of atrophoderma vermiculatum is challenging.MAIN OBSERVATIONS: We report the case of a 10-year-old boy, diagnosed as atrophoderma vermiculatum. The boy underwent two 6-month treatment courses with isotretinoin at a dose of 0.50mg/kg/day, which resulted in partial remission of the lesions. Even though the disease did not completely regressed, isotretinoin stopped atrophy progression and improved cosmetic appearance of the skin.CONCLUSIONS: In this case of a child with atrophoderma vermiculatum isotretinoin stopped atrophy progression and improved cosmetic appearance of the skin. No adverse events were observed. However, always, when applying isotretinoin in children, it has to be considered that, apart from possible common adverse events, this treatment may promote premature epiphyseal plate closure and may stop normal growth in some children.