This study was undertaken to determine the prevalence of molar incisor hypomineralization (MIH) in Syrian children and to provide information about clinical patterns and severity of MIH lesions. A sample of 1138 children aged 8–11 years was recruited for this cross-sectional study. The diagnosis of MIH was made using the criteria of the European Academy of Paediatric Dentistry (EAPD) and the MIH/HPSMs short charting form was used to score the index teeth. The results showed that the prevalence of MIH in Syrian children was 39.9%. Demarcated opacities were the most prevalent pattern of MIH defects on Permanent first molars (PFMs) and permanent incisors (PIs).Spearman rank correlationshowed that the mean number of PIs and HPSMs with MIH increased when the number of affected PFMs was increased (P< 0.001).Chi-squaretest resulted that girls showed a higher number of severe PFMs than boys did with a statistically significant difference (x2= 133.1,P< 0.05). Moreover,Chi-squaretest showed that the number of severe PFMs is higher than the number of severe PIs with a statistically significant difference (x2= 54.9,P< 0.05). In addition, the mean dmft/DMFT index in children with MIH was found to be significantly higher than children without MIH (P< 0.05). The findings emphasize the need for early identification and management of MIH in children to prevent adverse effects on their oral health.