A case of a botryoid odontogenic cyst of the globulomaxillary area between the right upper lateral incisor and the canine presenting as an apical periodontal cyst was reported. The cyst showed an unilocular radiolucency with a well delineated hyperostotic border. Histologic examination revealed multiple cysts lined by one or two-cell layers, some areas demonstrated a bud-like thickening of the epithelium. Clear cells were also conspicuous, but devoid of inflammatory reaction in the cystic wall. Some considerations regarding differential diagnosis, histogenetic and biologic behaviour of the lesion were discussed.