Ekman-Westborg and Julin is a trait that shows multiple macrodontia and multituberculism affecting only the teeth with no other anomalies (E-WJ). The aim of this report is to present a case which appears to manifest all the clinical signs of the E-WJ trait including odontoma formation. A 18-year-old girl with gingival inflammation particularly in the maxillary insicor area was referred to the authors' department. Panoramic, periapical and cephalometric radiographs were examined and complex odontoma associated with unerupted maxillary permanent lateral incisors was revealed. Intraoral examination revealed anterior crossbite, Angle Class III type malocclusion with mandibulary prominence and macrodontia of teeth 37, 12, 11 and 21. The patient was accepted as a new sporadic case of E-WJ. More case reports are needed to elucidate the causes and pathogenesis of this condition.