Background: In August 2021, three female semiconductor workers applied for occupational disease compensation due to congenital diseases diagnosed in their children: patient A (immunoglobulin A nephropathy, vesicoureteral reflux, renal agenesis), patient B (renal agenesis, esophageal atresia with tracheoesophageal fistula), and patient C (congenital megacolon). The Occupational Safety and Health Research Institute (OSHRI) initially assessed the relatedness of these conditions to occupational exposure as low. However, the Occupational Disease Adjudication Committee of the Korea Workers’ Compensation and Welfare Service (KWCWS) overturned this assessment, officially recognizing these cases as occupational diseases in March 2024—the first such recognition in South Korea.Case presentation: The mother of Patient A worked in optical processes for 9 years, the mother of patient B worked in diffusion processes for 10 years, and the mother of patient C worked in molding and inspection processes for 7 years. Their jobs involved exposure to benzene, organic solvents, pyrolysis products, ionizing radiation, and X-rays. All three women continued working during pregnancy, and none had a family history of related illnesses. Conclusions OSHRI estimated that the occupational exposure levels of these workers were low and noted insufficient reproductive research focused on congenital anomalies in the Korean semiconductor industry before 2010. However, KWCWS cited frequent miscarriages among female semiconductor workers as indirect evidence of an increased risk of congenital anomalies. KWCWS ultimately concluded that substantial evidence supports an association between occupational exposure and congenital diseases in the children of female semiconductor workers. This case series highlights a landmark recognition of occupational disease related to congenital anomalies in the semiconductor industry, emphasizing the need for further reproductive health research and improved worker protections.