Objective: The objective of the present study was to assess the nutritional intake and its relation to growth of children diagnosed with Phenylketonuria between the ages of 1.2-8 years. Materials and Methods: 8 participants were enrolled in this Cross- sectional study and their nutritional intake, growth was assessed using a dietary survey, stadiometer and digital weighing scale. Out of the 8 participants- 5 were girls and 3 were boys. The study participants were enrolled based on their disease condition using purposive sampling and an informed consent was obtained from the parents of these participants. The study was continued for a period of 4 months at Mediscan Systems, Department of Genetics, Chennai, India. Both inferential and descriptive statistics was used to quantify and digest the study parameters. Results: The study results suggested that 85% of the study participants were consuming an energy and protein deficient diet which was below 60% of the Recommended Dietary Allowance (RDA). Also, 80% of the participants had a lower than 10th percentile of Mid-Arm Circumference (MAC). About 75% study participants ranged between -2 to -3SD in terms of their weight. 90% participants attained a height of about -2 SD. The BMI/Weight for height ranged between -2 to -3 SD for 69% of the study population. Conclusion: This cross- sectional study highlights that there may be an influence of the nutritional intake on the nutritional status of the children diagnosed with PKU.