Congenital absence of portal vein is a rare anomaly that results from aberrant venous development in early embryonic life. The intestinal and splenic venous drainage bypass the liver and may drain directly into inferior vena cava or the left renal vein or the left hepatic vein. This rare anomaly is commonly associated with other congenital malformations and generally limited to females. We describe a rare case of aberrant portal vein development with congenital portocaval shunt (end-to-side) in a 3.5-year male child associated with cardiac defects (atrial and ventricular septal defects), skeletal deformities (flexion deformity and clinodactyly of digits and toes), and lichen planus with café au lait macules of skin.