A 3-month-old male infant was found to have a protruding mass with irregular contour at the root of his nose since birth. The mass protruded more when he cried. It was diagnosed and treated as a hemangioma by laser surgery in another hospital. However, the lesion recurred 1 week later. Reviewing the previous computed tomographic study of the brain, it revealed that the protruding mass communicated with the brain. Sincipital encephalocele was diagnosed, and craniotomy for excising the encephalocele and repair of the dura were undertaken successfully. We report this patient with sincipital encephalocele that was misdiagnosed and treated as a vascular malformation on the face. Fortunately, there was no complication. The authors stress the importance of accurate diagnosis of a lesion near the craniofacial junction to avoid unnecessary surgery and possible catastrophes.