Background: Developmental atlantal stenosis with myelopathy (DASM) in adults is a rare disease that only sporadic cases have been reported over the years. C1 laminectomy (C1L) is one of the most common operations for its treatment. However, as an open surgery, it has shortcomings such as large trauma and slow postoperative rehabilitation, and minimally invasive spine surgery (MISS) offers alternative treatment options with advantages. MISS instruments expand the technical capabilities of surgeons, which allows a safer and more effective therapeutics for difficult and complicated diseases. This case report presents a new minimally invasive approach; percutaneous full endoscopic C1 laminectomy (PFEC1L), for the treatment of DASM, and to consolidate the current literature on the condition to summarize its etiologies, clinical manifestations, diagnostic criteria, surgical management, and prognoses. Case Description: The patient in Case 1 presented with neck pain and numbness and weakness in the limbs. The patient in Case 2 presented with numbness in the extremities and the patient in Case 3 presented with bilateral hand numbness and left lower limb weakness. They were all diagnosed with DASM and underwent PFEC1L treatment to maintain the enlargement and decompression of the atlantal canal, which achieved favorable outcomes without complications during the postoperative follow-up visit. Conclusions: DASM is rare but potentially dangerous. Its diagnosis is made based on clinical manifestations combined with radiological imaging examinations, especially computed tomography (CT) scan and magnetic resonance imaging (MRI). While C1L is the most common surgical method, PFEC1L is a new feasible and safe therapeutic option with comparable good outcomes and the advantage of being minimally-invasive. To our knowledge this is the first report that PFEC1L was applied for DASM treatment.