We report a case of ileal atresia presenting with massive chylous ascites and hydrocele in a neonate. A male neonate was born at 38 weeks of gestation with a weight of 3,360 grams. The antenatal ultrasonography performed at 37âºâ¶ weeks of gestation showed ascites and huge bilateral hydrocele. At birth, he manifested hypotonia, a massively distended abdomen and huge bilateral hydrocele. Exploratory laparotomy was performed on the day of birth. A large amount of chyle (approximately 200 mL) was found in the peritoneal cavity between the bowel loops. An additional 120 mL of chyle was removed from both scrotal sacs. The color of chyle was ivory yellow with whitish debris. Ileal atresia had occurred 7 cm above the ileocecal valve. The proximal small bowel was disconnected, and the distal small bowel loop ended in a blind loop. Lymphatic drainage from that cross section of the proximal small bowel might be a cause of chylous ascites. Segmental small bowel resection and double barrel ileostomy was performed. We described this case with a review of the literature.