Nutcracker esophagus and hypertensive lower esophageal sphincter (LES) are primary esophageal motility disorders. Nutcracker esophagus have demonstrated distal esophageal contraction amplitude that exceeds the normal range (>160mmHg), without association abnormalities of the esophageal contraction wave, or lower esophageal sphinter relaxation. The criteria for diagnosing hypertensive LES take the mean LES pressure >45mmHg, LES relaxation >75% and normal peristalsis, which is a poorly characterized motility disorder associated with chest pain and dysphagia. We experienced a case of nutcracker esophagus combined with hypertensive LES in a 3-year- old girl who presented with projectile vomiting immediately after eating solid foods. After she had taken barium esophagography and esophageal endoscopy, we suspected she had esophageal motor disorder. Esophageal manometric findings show abnormal high pressure of LES (mean LES pressure, 52.9 mmHg), abnormal high amplitude of lower esophagus more than 320 mmHg, normal esophageal perisaltic movement and normal LES relaxation. After pneumatic dilatation, now she can eat semisolid foods. This case may be the first case of nutcracker esophagus combined with hypertemsive LES in children.