Objective: The aim of our work was to access the epidemiological and clinical aspects of congenital hydrocephalus in the pediatrics department of Gabriel Touré teaching Hospital Bamako. Methods: We conducted a retro and prospective study that ran from January, 1st 2018 to July, 30, 2019. All newborns of 72 hours of life or less with hydrocephalus confirmed by sonography or CT scan were enrolled in this study. Results: During the study period, 5416 patients were hospitalized in the neonatology department. Among them, 39 patients (0.72%) presented congenital hydrocephalus and congenital infectious causes accounted for 25.6%. The sex ratio (M/F) was 1.78. Mothers were housewives and not educated in 79.5% and 64.1% respectively. The parents resided outside Bamako in 61.5% of cases. Prenatal consultations were conducted in 32 patients (82%). Consanguinity between the 2 parents was present in 38.5%. The antenatal diagnosis was made in 8 patients (20.5%). Vaginal delivery was the main mode of birth (67%). The average birth weight was 2930 g (1000 to 5400 g) and the average head circumference was 37.82 cm (26 -55 cm). In 87.2% of cases, newborns were eutrophic. The main clinical signs were bulging anterior fontanel (46.2%), sunset gaze (20.5%), prominent forehead (48.7%), reduced face (20.5%), enlarged cranial sutures (43.6%), macrocrania (25.6%). Transfontanellar ultrasound and CT scan were performed in 51.