Pyknodysostosis is a rarely encountered disease. It is characterized by abnormal facial development, fragility of the bones with relatively mild trauma, cranial abnormalities including widely opened sutures and fontanels frontal bossing, wrinkled skin, finger and nail abnormalities. In the literature, pyknodysostosis was reported only as case reports. Here, we describe a pyknodysostotic child with a bilateral parietal epidural hematoma, caused by a relatively mild head trauma. He was operated on, and the hematoma was evacuated through two different parietal craniotomies. We reported this case, since pyknodysostosis is a rare disease, and our case is the first one who had a surgical intervention as well as being the first with an epidural hematoma in pyknodysostosis.

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