This case report presents the epidemiology, symptomathology, pathogenesis and treatment of a melanotic neuroectodermal tumour of infancy. MNTI is a rare tumour that is seen usually at or around birth. It is usually localised in facial region. The tumour originated from medial part of alveolar process of maxilla. Presented case developed in maxilla of four months old girl. Excision of a tumour with a dental germ 61 was performed. Despite MNTI shows benign hystological character it has a high recurrence potential. Two months after first operation during routine follow up control, there was a tumour in oral cavity that was suspected to be relapse. Patient undergone second prodecure in general anesthesia. Tumour was excised with radical margins. Due to complex funcitional and aesthetic character of facial region patients need specialistic treatment and rehabilitation of masticatory organ. Frequent presence of dental germs in the mass of tumour often causes malocclusion.