Object The aim of this study was to examine the distribution of intracranial arachnoid cysts in a large and unselected patient population with special emphasis on sidedness and sex distribution. Methods In total, 299 patients with 305 arachnoid cysts were studied. These patients were consecutively referred to our department during a 20-year period from a well-defined geographical area with a stable population. Results There was a strong predilection (198 patients [66.2%]) for intracranial arachnoid cysts in the temporal fossa. Forty-two patients had cysts overlying the frontal convexity, 36 had cysts in the posterior fossa, and 23 patients had cysts in other, different locations. Of 269 cysts with clearly unilateral distribution, 163 were located on the left side and 106 on the right side. This difference resulted from the marked preponderance of temporal fossa cysts on the left side (left-to-right ratio 2.5:1; p < 0.0001 [adjusted < 0.0005]). For cysts in the cerebellopontine angle (CPA), there was preponderance on the right side (p = 0.001 [adjusted = 0.005]). Significantly more males than females had cysts in the temporal fossa (p = 0.002 [adjusted = 0.004]), whereas in the CPA a significant female preponderance was found (p = 0.016 [adjusted = 0.032]). For all other cyst locations, there was no difference between the 2 sexes. Conclusions Arachnoid cysts have a strong predilection for the temporal fossa. There is a sex dependency for some intracranial locations of arachnoid cysts, with temporal cysts occurring more frequently in men, and CPA cysts found more frequently in women. Furthermore, there is a strong location-related sidedness for arachnoid cysts, independent of patient sex. These findings and reports from the literature suggest a possible genetic component in the development of some arachnoid cysts.