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MOMO syndrome is a very rare disorder that consists of macrosomia, obesity, macrocephaly, and ocular abnormalities. Very little is known about the implications of this syndrome as only 10 cases have been previously reported in the literature. Previous publications have observed multiple associations with this syndrome, such as intellectual disability, delayed bone age, and skeletal abnormalities. We report the first case of a patient with MOMO syndrome who presented with ovarian torsion from a large serous cystadenoma of the right ovary.