Primary melanotic progonoma of skull

Melanotic progonoma is a rare tumour that generally arises in the maxilla during the first year of life. The involvement of cranial vault bones is extremely rare. The authors report the case of a 7-month-old female infant admitted for a congenital right parieto-occipital bone tumour. The radiological examination showed a spicular opacity pattern. The surgical resection was complete. We highlight the rarity of this neoplasm and discuss epidemiological, clinical, radiological and therapeutical characteristics of this pathology