Obstructive sleep apnea syndrome (OSAS) is commonly seen in patients with neurological disorders, such as amyotrophic lateral sclerosis (ALS), especially later in disease course when diaphragmatic dysfunction and respiratory compromise becomes evident. The gold standard therapy of OSAS is the noninvasive mechanical ventilation (NIMV). The mainstay treatment for patients with ALS and respiratory disturbances is also accepted as NIMV to prolong life expectancy. Diaphragm pacing stimulation (DPS) has recently been introduced and suggested to be beneficial in patients with ALS. Here we report a patient with ALS, in whom OSAS was completely resolved upon DPS intervention. With more common application of diaphragmatic pacing in ALS patients with respiratory involvement will offer a supportive treatment rather than traditional palliative care.