Pai syndrome (PS) is a rare regional developmental defect of the face, mainly characterizedby the variable association of midline cleft of the upper lip (MCL), facial cutaneous lipomas,midanterior alveolar process and nasal polyps and intracranial (pericallosal) lipomas. Its entire clinicalspectrum is still poorly delineated and the etiology remains unknown. Very few cases of Pai syndromediagnosed prenatally have been described. Pai syndrome is usually diagnosed at birth.Differential diagnoses include Loeys-Dietz syndrome, Oculocerebrocutaneous syndrome, frontonasaldysplasia and Goldenhar syndrome, along with chromosomal anomalies. In this case report4-year-old female patient with nasal and pericallosal lipoma is presented and the clinical and radiologicalfeatures of Pai syndrome were discussed with the literature review.