Background: The neurocutaneous syndromes are a group of diseases where characteristic skin lesions are seen in association with abnormalities of the central nervous system, and by consequence are at risk of developing epilepsy and other comorbidities. Objectives: The objective of this study is to study clinical presentations and diagnoses among Sudanese children with neurocutaneous syndrome, attending outpatient clinic of Soba University Hospital, 2015. Patients and Methods: This is a cross-sectional hospital-based study. Fifty children with neurocutaneous syndrome were included in the study aged between 6 months to 15 years attending the hospital from April 2014 to April 2015. The data was collected by designed data collection sheet and detailed skin examination was done and reviewed by dermatologist. Results: The mean age of the whole study group was 4.45 ± 1.96 years. Most of study group were in the age group between 1-5 years constituting 36% of total population of the study. Thirty-seven patients (74%) were males and 13 (26%) patients were females. About 24% of patients presented complained of skin lesions from total patients, the most common skin lesion is ash leaves. Conclusion: Neurocutaneous syndromes had multiple clinical presentations. Most of the patients with neurocutaneous disorders had convulsions, which was commonest presentation for those children; generalized convulsion was the commonest among the studied group. Tuberous sclerosis was the most frequent type of the neurocutaneous disorders in our patient clinic. Dermatological examination is important, for early detection of neurocutaneous disorders.