Osteopathia striata with cranial sclerosis is a skeletal dysplasia that presents a variety of clinical symptoms along with typical radiological characteristics. While there are a number of distinct skeletal dysplasias to take into account when making a differential diagnosis for osteopathia striata with cranial sclerosis, none of them exhibit the simultaneous calvarial thickening and longitudinal striations of the long bones as the aforementioned disease. Here we report a rare case of a 25-year-old male patient who suffered from cranial nerve deficiencies due to encroachment of the neural canals by sclerosis with involvement of the optic and acoustic nerves. This paper demonstrates the process of investigating a rare progressive genetic disease and highlights the differential diagnostic considerations. This case teaches us the radiological characteristics of osteopathia striata with cranial sclerosis and displays current imaging techniques. The article also summarizes the diseases with cranial sclerosis and sclerotic metaphyseal striations.  Kulcsszavak: skeletal dysplasia, metaphyseal striations, hyperostosis, photon-counting detector CT, MRI