Background: Menkes disease is a rare, X-linked recessive disorder of the ATP7A gene, a copper transporter; resulting in systemic copper deficiency. The deficient function of copper-dependent enzymes manifests clinically with failure to thrive, seizures, hypotonia, coarse hair, connective tissue abnormalities, and neurodegeneration. Cerebral arteries are often elongated, tortuous, and fragile. Methods: This case report was prepared using the patient’s hospital chart, and a review of the literature undertaken using PubMed. Our case was subsequently compared and contrasted to known Menkes’ literature. Results: We present the case of a 2 month old male with Menkes disease who presented with new seizure onset in the setting of a Grade III intraventricular hemorrhage with hydrocephalus. He deteriorated into status epilepticus, and palliative care was instituted. On autopsy, pronounced tortuosity of his cerebral vasculature was noted, as well as a bilaterally cystic brain with an organizing hemorrhage on the ventral surface of the brainstem. Conclusions: Although Menkes disease often presents with seizures, neurologic deterioration, and abnormal cerebral vasculature; the quick demise subsequent to an intraventricular hemorrhage is somewhat unusual and discussed.