Objective To assess the safety and efficacy of endovascular treatment for basilar tip aneurysms associated with moyamoya disease. Methods Seven patients with basilar tip aneurysms associated with moyamoya disease were treated by endovascular embolization in our department between Oct. 2006 and Jul. 2013. Two patients were treated by pure coiling and the others were treated by stent-assisted coiling. The immediate angiographic result was evaluated by Raymond scale. Postoperative angiographic and clinical follow-ups were obtained in all the 7 cases, and the clinical outcomes were evaluated by the modified Rankin score (mRS). Results All the 7 patients successfully received endovascular embolization without procedure-related complications. Immediate angiographic results showed complete occlusion in 3 cases, neck residual in 2, and partial occlusion in 2. Postoperative angiographic follow-ups were obtained for a mean of (10.4±3.5) months, with complete occlusion found in 6 patients and stable in 1 patient. No progressive thrombosis or in-stent occlusion were detected by follow-up angiograms in the stent group. Clinical follow-ups were obtained for a mean of (21.1±15.8) months and all the patients reported good outcomes (mRS 0-2). Conclusion Our data suggest that endovascular embolization is a safe and efficient treatment for basilar tip aneurysms associated with moyamoya disease. Stent-assistance might be a safe and effective option for the large or wide-necked aneurysms, though the long-term safety still remains to be confirmed.