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Craniopharyngiomas (CPs) are rare benign epithelial tumours. Brain arteriovenous malformations (AVMs) are also rare lesions occurring in young adults. The appearance of both these lesions in the same patient is rare. A 42-year-old patient presented with headaches for 3 months and a progressive decrease in his visual acuity. Bitemporal hemianopsia was detected in the visual field. Magnetic resonance imaging (MRI) revealed a tumour with cystic and solid components located in the suprasellar region and AVM in the right temporal lobe. AVM (Martin–Spetzler grade III) was visualised using digital subtract angiography (DSA), which was fed from the right middle cerebral artery and drained through the sigmoid sinus via the inferior petrosal sinus. The patient was operated with enlarged right frontotemporal craniotomy. AVM nidus was totally removed at the first operation. Embolisation was not preferred before the AVM surgery. After 3 days, sylvian dissection was performed using the same craniotomy. The tumour was completely removed via the carotid cistern by making sharp dissection from the infundibulum. Post-operatively, the patient showed normal neurological examination and significant improvement in his visual field examination. There was no residual/recurrent tumour or AVM on contrast-enhanced MRI and DSA at post-operative 6 months. Histopathological examination revealed AVM in the first operative material and papillary-type CP in the second. The coexistence of these two rare pathologies has previously been reported in only one patient. This is the first case of surgical resection of CP and AVM using the same craniotomy.