Loeys-Dietz syndrome (LDS) is a connective tissue disorder with associated systemic vasculopathies including intracranial arterial aneurysm formation and dissections. LDS is a relatively less well-known entity compared with other connective tissue disorders, such as Ehlers-Danlos or Marfan syndrome, and consequently experience in the management of the associated intracranial aneurysms is suboptimal. We present a case of surgical clipping of a middle cerebral artery aneurysm in a patient with LDS. A 46-year-old female with LDS (type III) was found to have a right middle cerebral artery (MCA) bifurcation aneurysm following vascular screening. The decision was made to surgically clip the aneurysm after consultation in our neurovascular multidisciplinary team meeting. A standard right pterional craniotomy was performed and the aneurysm was secured with 2 straight Sugita clips. The temporal M2 branch was noted to be thin walled and this prompted application of the second tandem clip, rather than risk re-positioning the initial clip. In our case, the MCA aneurysm neck was robust enough to take a clip without any complications, and therefore we suggest that the presence of LDS is not an absolute contra-indication to perform open craniotomy and clipping.