Cranial hemophilic pseudotumor (cHPT) is a very rare disease, which is easy to misdiagnose. It is also difficult to manage such patients. We reported the first case of occipital cHPT. Case presentation: Here, we presented a rare case of an occipital bone mass in a 3-year-old boy who was diagnosed with hemophilia A. The mass was misdiagnosed as an aneurysmal bone cyst by pathological examination. After resection, the patient underwent one-stage cranioplasty. However, the patient was admitted again for hematoma caused by an invasive procedure. A second surgery and one-stage cranioplasty were performed at the same time. A follow-up 3 months after discharging showed the patient was uneventful, and the titanium mesh was well fixed. Conclusion: The diagnosis of cHPT requires the combining of history, radiological examination, and pathological examination. Resection is the best choice for symptomatic cHPT. Replacement treatment and less invasive treatment can make perioperative management safer. One-stage cranioplasty for resection of an occipital cHPT can improve the quality of life.