Malignant chondroid syringoma is a mixed cutaneous tumor, with epithelial and mesenchymal components, which compromises principally the trunk and extremities. This lesion is quite rare, with few cases related in the literature and no publications demonstrating its involvement of the central nervous system. Histologically, owing to its mixed origin, it represents a lesion that is difficult to recognize, often being confused with basocellular carcinoma. We report the case of a female patient, carrier of malignant chondroid syringoma in the occipital region, with invasion of the central nervous system, who was submitted to surgical excision of the lesion at our service. We also made a brief revision of the literature on the theme.

We observed that, in the study population, EDH appears more often in males, in the fourth decade of life, and is more related to falls. On admission, GCS was observed between 13 and 15 and it is appropriate to mention the involvement of the temporo-parietal region in most cases. We believe that knowledge of the epidemiology of traumatic epidural hematoma can assist in developing public health measures aimed at prevention and early identification of this disease in the population.

The present case is the ninth diagnosed during the prenatal period and the literature is controversial on the management of this rare tumor. The complete excision of the lesion using the microsurgical technique is the gold standard treatment for these patients; however, there are many factors that limit this approach in neonates. Usually, the resected tumors were smaller than 6 cm. Tumors larger than 8 cm have a worse prognosis, with a short survival time.