Dranje syndrome affects the physical development of children, leading to symptoms such as peculiar facial features and abnormal mental development. Dranje syndrome multiple congenital developmental anomalies syndrome, an autosomal dominant disorder, is mostly a de novo mutation. The defective gene can be inherited from a parent, but the vast majority of patients have spontaneous mutations. The main features of Dranje syndrome are a peculiar face with thick eyebrows, thin lips, downward sloping corners of the mouth, a narrow forehead, bulging eyes, and a high palatal arch. Intrauterine and postnatal growth retardation, hirsutism, and upper extremity repositioning defects are present, and common organ abnormalities include ptosis, intestinal malrotation, hypospadias, and pyloric stenosis. Often, severe mental retardation is present in affected children. A good premarital physical examination before the gestational period is an effective diagnostic means of prevention. Regular checkups should be performed during pregnancy, and if abnormalities are detected, it should be clarified whether the pregnancy should be terminated.