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Spontaneous thrombosis of a vein of Galen aneurysmal malformation (VOGM) is rare. We describe a 2-month-old patient with a patent VOGM and hydrocephalus, also confirmed patent at 6 months, but with subsequent lack of filling on preembolization catheter digital subtraction angiography (DSA) at 9 months’ age. Due to the presence of T1- and T2-bright signal, noncontrast T1-weighted images (T1WI), T2-weighted images (T2WI), two-dimensional (2D) time-of-flight (TOF) magnetic resonance venography (MRV), and postcontrast T1WI were ambiguous for patency. However, subtracting the pre- from the postcontrast MRV images confirmed closure compared to subtracted images at 6 months’ age. The factors contributing to thrombosis were likely a combination of a disproportionately small straight sinus, ventriculostomy, and contrast medium from DSA.