Background: Weill-Marchesani syndrome is a rare condition with ocular and systemic manifestations. Common ocular signs are microspherophakia, ectopia lentis, and secondary glaucoma. The glaucoma is usually treatable through medical or surgical management. The most-common arteriovenous malformation (AVM) with ocular effects is the carotid-cavernous sinus fistula. It is very uncommon to observe ocular complications from an AVM in the area of the straight sinus and vein of Galen. AVMs in any location are unusual with Weill-Marchesani syndrome.

Methods: We present a case of severe asymmetric glaucoma in a Weill-Marchesani patient with an AVM in the area of the straight sinus and vein of Galen. The patient demonstrates the known clinical signs of Weill-Marchesani syndrome: short stature, microspherophakia, lens subluxation, and secondary glaucoma. An AVM is diagnosed with corresponding monocular proptosis and asymmetric glaucoma. The patient's serious systemic health problems and the location of the AVM make treatment difficult.

Conclusions: This case documents the very unusual occurrence of unresponsive glaucoma secondary to an AVM not located in the cavernous sinus. The Weill-Marchesani syndrome may be partially responsible for the glaucoma. Patients with Weill-Marchesani syndrome require early diagnosis in order to treat serious ocular and life-threatening systemic complications.