Achromobacter xylosoxidans (AX), also called alcaligenes xylosoxidans, is an aerobic, non-fermenting mobile, gram-negative bacillus which was first isolated in an otorrhea samples in 1971. Infections with these species are quite rare and have often been described in immunocompromised and in premature infants. However, very few cases of meningitis related to AX have been reported in the literature. The authors report a new case of meningitis due to AX in a 45-day-old female infant with polymarformative syndrome meningitis was confirmed by a cyto-biochemical analysis and culture of the cerebrospinal fluid and was treated by antibiotherapy. Hydrocephalus was managed initially with external ventricular drainage followed by a ventriculoperitoneal shunt after rigorous cerebrospinal fluid (CSF) sterilization, with good clinical and radiological outcomes. The prompt and adequate antibiotic adjustment following bacterial isolation has been shown to rapidly modify the clinical outcomes.