A 4-year-old girl presented repeatedly with a complicated occipital mass, which was erroneously treated as a pyogenic granuloma. Imaging performed before a planned surgical resection detected an underlying intraoccipital dermoid with a sinus tract to the skin surface and extension into the posterior fossa. This case highlights the value of high-resolution computed tomography imaging for depiction of anatomic details and the value of magnetic resonance imaging for differential diagnosis and surgical management. A comprehensive literature review of intraosseous dermoid cyst and detailed discussion of the differential diagnoses are provided.