BackgroundChondroblastoma is a rare, benign bone tumor originating from immature chondrocytes, typically found in the epiphyseal plates of long bones. Its occurrence in the skull, particularly the occipital bone, is extremely rare.Case presentationWe report a 10-year-old girl presenting with paroxysmal dizziness, occipital pain, and a palpable mass. Imaging studies revealed an osteolytic lesion in the occipital bone with a multiloculated appearance, indicating a concomitant aneurysmal bone cyst. The patient underwent craniotomy with complete tumor excision. Postoperative follow-up showed no neurological deficits or complications and MRI confirmed no recurrence.ConclusionThis case highlights the rare presentation of occipital bone chondroblastoma and emphasizes the importance of early surgical intervention for positive outcomes in pediatric patients.