The association between idiopathic intracranial hypertension (IIH) and obstructive sleep apnea syndrome (OSAS) constitutes an interesting point of discussion regarding clinical approaches as well as the underlying pathophysiological mechanisms. Here, the case of a 42-year-old female with malignant IIH and OSAS is illustrated. Initially, the patient was treated with lumboperitoneal shunt surgery. However, OSAS surgery provided total recovery from the recurrent symptoms developing secondary to lumboperitoneal shunt malfunction. The authors point out the importance of identifying OSAS as a crucial causative agent in some IIH subtypes. Future studies are warranted to clarify this underestimated association.