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AIM: To assess the efficacy of treatment using a minimally invasive approach (selective removal of carious tissue, restoration and preventive strategies) in immature permanent molars with MIH. DESIGN: A total of 281 patients, aged 6-8 years, with carious lesions (ICDAS 5-6), severe MIH, and incomplete root formation (one tooth/patient) were included. After clinical and radiographic examinations, selective carious tissue removal was performed, and the teeth received interim restoration for 6 months and were then restored with composite resin. Clinical and radiographic follow-up was undertaken, 6, 12, 18, and 24 months. A protocol of preventive oral care measures was established and repeated at each follow-up, including diet counselling, oral hygiene instruction, dental plaque control, and topical application of fluoride varnish containing CPP-ACP. All clinical procedures and evaluations were done by a single operator. RESULTS: Clinical and radiographic success was observed 24 months after treatment in 96.8% of the cases. Failures were due to enamel fracture at restoration margins, resulting in pulpitis and absence of apex closure. CONCLUSION: Selective removal of carious tissue, interim, and subsequently definitive restoration, combined with home and professional preventive measures, maintained marginal integrity of restorations in immature permanent molars with severe MIH, confirmed by pulp vitality and occurrence of apexogenesis.