Vitamin K Deficiency Bleeding (VKDB) is seen rarely and it may be life-threatening during early infancy. VKDB is classified according to bleeding times as early (0-1 day), classical (1-7 days) and late (8 days to 6 months). Undiagnosed cholestasis is usually present in the late type in which the bleeding commonly starts in 3-8 weeks. Niemann Pick disease is a type of lysosomal storage disease associated with special storage cells which are very similar to foamy histiocytes and occurs due to sphingomyelin build-up. It’s known that cholestasis may develop and go away on its own among new-borns in the types of A or C of the disease. PT and PTT were prolonged in a 2-month-old patient who presented with umbilical bleeding which had happened three times since the first month. Cholestasis, which is a risk factor for late type VKDB, was investigated and diagnosed. Bleeding did not repeat after Vitamin K administration. The patient was diagnosed with Niemann-Pick Type A by showing a new homozygous mutation in SMPD1 gene after advanced investigations because of liver and spleen enlargement despite cholestasis was regressing during follow-up. This article presents a rare case diagnosed with Niemann-Pick Type A for the first time presented with umbilical bleeding due to Vitamin K deficiency.