Marcus Gunn jaw winking (MGJW) is an uncommon entity and is associated with congenital ptosis. It is a neurogenic congenital ptosis, which is also called the Marcus Gunn phenomenon, trigeminal-oculomotor synkinesis, or pterygoid-levator synkinesis. Congenital ptosis can be associated with MGJW syndrome, blepharophimosis syndrome, and monocular elevation deficiency (MED). MED is a condition where there is a unilateral congenital abnormality in the elevation of the eye in abduction and adduction. The MGJW phenomenon, congenital ptosis, and double-elevator palsy may be associated with and represent a congenital misdirection syndrome. Together, it can be challenging, and surgery is recommended in severe cases, depending on the degree of ptosis and jaw winking. We hereby want to bring to light one such case of a 14-year-old female with congenital ptosis, MGJW, and double-elevator palsy and want to highlight how both MGJW and double-elevator palsy are both parts of the same disease spectrum and how such cases can be treated.