Background: Neurofibromatosis is a genodermatosis which is common worldwide without any age, gender or racial preference. Its effects are multi-systemic, requiring a multidisciplinary approach in management to reduce the morbidity and mortality associated with it. Aim: This study reviewed the epidemiology and management of patients with neurofibromatosis in the centre. Method: This study is a retrospective cross sectional study of neurofibromatosis cases seen over a ten year period within January 2006- December 2015 in the University of Port Harcourt Teaching Hospital Alakahia, in Rivers State, Nigeria Results: There were 32 patients with clinically diagnosed neurofibromatosis type 1 with an average incidence rate of 0.54%. There was slight female preponderance in the study. The highest incidence was seen in the second decade of life. The duration of the skin lesions before presentation to the dermatology clinic ranged from 1-31 years. Neurofibromas were seen in all patients of whom plexiform neurofibromas were seen in about 30% of the patients. The commonest site on the body which the lesions started from was the trunk (30%), followed by the face (10%). In terms of spread, the face (46.7%) was most affected, followed by the trunk (36.7%), the upper and lower limbs (33%) respectively. Axillary freckles and café au lait spots were the second commonest clinical features. Conclusion: Neurofibromatosis type 1(NF1) is of public health importance because it causes body distortion leading to social stigmatization. It can be mistaken for an infectious disease like leprosy or tuberculosis. It is more commonly seen than neurofibromatosis type 2 (NF2) in our region. Its diagnosis can be made clinically and by genetic testing. Complications are diverse and management is multidisciplinary.